Compounds > methylmalonic acid
Page last updated: 2024-11-04

methylmalonic acid

Description Research Excerpts Clinical Trials Roles Classes Pathways Study Profile Bioassays Related Drugs Related Conditions Protein Interactions Research Growth Market Indicators

Description

Methylmalonic Acid: A malonic acid derivative which is a vital intermediate in the metabolism of fat and protein. Abnormalities in methylmalonic acid metabolism lead to methylmalonic aciduria. This metabolic disease is attributed to a block in the enzymatic conversion of methylmalonyl CoA to succinyl CoA. [Medical Subject Headings (MeSH), National Library of Medicine, extracted Dec-2023]

methylmalonic acid : A dicarboxylic acid that is malonic acid in which one of the methylene hydrogens is substituted by a methyl group. [Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Cross-References

ID SourceID
PubMed CID487
CHEMBL ID1232416
CHEBI ID30860
SCHEMBL ID27766
MeSH IDM0013646

Synonyms (75)

Synonym
bdbm50038341
CHEMBL1232416
alpha-methylmalonic acid
CHEBI:30860 ,
methylpropanedioic acid
nsc25201
1,1-ethanedicarboxylic acid
nsc-25201
propanedioic acid, methyl-
2-methylmalonic acid
isosuccinic acid
malonic acid, methyl-
wln: qv1vo1
einecs 208-219-5
ai3-19472
brn 1756084
nsc 25201
inchi=1/c4h6o4/c1-2(3(5)6)4(7)8/h2h,1h3,(h,5,6)(h,7,8
C02170
methylmalonic acid
516-05-2
methylmalonate
methylmalonic acid, 99%
DB04183
MLS001335866
smr000857347
MLS001335865
methyl malonic acid
166A2535-D607-4D47-956D-8FC6A1EA3B08
propanedioic acid, methyl- (9ci)
2-methylpropanedioic acid
BMSE000398
FT-0655314
M0568
LMFA01170118
2-methyl-propanedioic acid
NCGC00247049-01
AKOS009157181
HMS2230A22
S3683
FT-0671897
unii-8ll8s712j7
4-02-00-01932 (beilstein handbook reference)
8ll8s712j7 ,
STL373471
1215348-94-9
propanedioic acid, 2-methyl-
BBL027393
SCHEMBL27766
SY006109
mfcd00002656
2-methyl-malonic acid
2-methylpropanedioicacid
DTXSID00199549
AC1639
gtpl9633
1,1-ethanedicarboxylate
methyl-propanedioate
isosuccinate
methyl-malonic acid
2-methylmalonate
methyl-propanedioic acid
methyl-malonate
CS-0027817
HY-103395
BCP22931
Q239598
STR03479
HMS3869I13
CCG-266070
methylpropanedioic acid;methylmalonate
2-methylmalonicacid
EN300-53151
2-methylmalonic acid 100 microg/ml in acetonitrile
Z787653408

Research Excerpts

Overview

Methylmalonic acidemia (MMA) is a rare and severe inherited metabolic disease typically caused by mutations of the methylmalonyl-CoA mutase (MMUT) gene. It is a neurometabolic disorder biochemically characterized by the accumulation of methylmalonic acids in different tissues, including the central nervous system.

ExcerptReferenceRelevance
"Methylmalonic acidemia is an organic acidemia caused by deficient activity of L-methylmalonyl-CoA mutase or its cofactor cyanocobalamin and it is biochemically characterized by an accumulation of methylmalonic acid (MMA) in tissue and body fluids of patients. "( Methylmalonic Acid Impairs Cell Respiration and Glutamate Uptake in C6 Rat Glioma Cells: Implications for Methylmalonic Acidemia.
Alberto-Silva, C; Carrettiero, DC; Costa, RT; Ribeiro, CAJ; Santos, MB, 2023)		3.8
"Methylmalonic acidemia (MMA) is a rare and severe inherited metabolic disease typically caused by mutations of the methylmalonyl-CoA mutase (MMUT) gene. "( Growth advantage of corrected hepatocytes in a juvenile model of methylmalonic acidemia following liver directed adeno-associated viral mediated nuclease-free genome editing.
Chandler, RJ; Chau, N; Gordo, S; Hoffmann, V; Liao, J; Ramesh, N; Venditti, CP; Venturoni, LE, )		1.81
"Methylmalonic acidemia is a neurometabolic disorder biochemically characterized by the accumulation of methylmalonic acid (MMA) in different tissues, including the central nervous system (CNS). "( Methylmalonic acid induces inflammatory response and redox homeostasis disruption in C6 astroglial cells: potential glioprotective roles of melatonin and resveratrol.
Bobermin, LD; de Souza Almeida, RR; Gonçalves, CA; Leipnitz, G; Parmeggiani, B; Quincozes-Santos, A; Souza, DO; Wajner, M; Wartchow, KM, 2022)		3.61
"Methylmalonic acidemia (MMA) is a severe inborn error of metabolism that is characterized by pleiotropic metabolic perturbations and multiorgan pathology. "( New insights into the pathophysiology of methylmalonic acidemia.
Head, PE; Meier, JL; Venditti, CP, 2023)		2.62
"Methylmalonic Acidemia (MMA) is a heterogenous group of inborn errors of metabolism caused by a defect in the methylmalonyl-CoA mutase (MMUT) enzyme or the synthesis and transport of its cofactor, 5'-deoxy-adenosylcobalamin. "( Biomarkers to predict disease progression and therapeutic response in isolated methylmalonic acidemia.
Catesini, G; Dionisi-Vici, C; Ferry, S; Gagné, J; Gebremariam, A; Hall, C; Manoli, I; Martinelli, D; McCoy, S; Pass, AR; Rizzo, C; Sacchetti, E; Sloan, JL; Spada, M; Van Ryzin, C; Venditti, CP, 2023)		2.58
"Methylmalonic Acidurias (MMAs) are a group of inborn errors of metabolism (IEMs), specifically of propionate catabolism characterized by gastrointestinal and neurometabolic manifestations resulting from a deficiency in the function of methylmalonyl-CoA mutase, methylmalonyl-CoA epimerase, and cobalamin metabolism. "( A False-Positive Case of Methylmalonic Aciduria by Tandem Mass Spectrometry Newborn Screening Dependent on Maternal Malnutrition in Pregnancy.
Brindisino, P; Cicalini, I; Consalvo, A; De Laurenzi, V; Dionisi-Vici, C; Gasparroni, G; Gazzolo, D; Pieragostino, D; Rizzo, C; Rossi, C; Semeraro, D; Valentinuzzi, S; Zucchelli, M, 2020)		2.3
"Methylmalonic acidemia is an inborn metabolic disease of propionate catabolism, biochemically characterized by accumulation of methylmalonic acid (MMA) to millimolar concentrations in tissues and body fluids. "( The Effect of Methylmalonic Acid Treatment on Human Neuronal Cell Coenzyme Q
Bennett, E; Boan, EJ; Hargreaves, IP; Heaton, RA; Philips, S; Proctor, EC; Turton, N, 2020)		2.36
"Methylmalonic acidemia is an inherited organic acid metabolic disease. "( A rare mutation c.1663G > A (p.A555T) in the MMUT gene associated with mild clinical and biochemical phenotypes of methylmalonic acidemia in 30 Chinese patients.
Chen, X; Chen, Y; Feng, J; Gong, Z; Gu, X; Han, L; Hu, H; Ji, W; Liang, L; Lu, D; Niu, T; Qiu, W; Shen, L; Shuai, R; Wei, H; Wu, S; Xu, P; Yang, C; Ye, J; Yu, Y; Zhan, X; Zhang, H; Zou, H, 2021)		2.27
"Methylmalonic acidemia is a rare metabolic disorder caused by the deficient activity of l-methylmalonyl-CoA mutase or its cofactor 5-deoxyadenosylcobalamin and is characterized by accumulation of methylmalonic acid (MMA) and alternative metabolites. "( Methylmalonic Acid Compromises Respiration and Reduces the Expression of Differentiation Markers of SH-SY5Y Human Neuroblastoma Cells.
Carrettiero, DC; da Costa, RT; de Almeida, RP; Dos Santos, MB; Ribeiro, CAJ; Silva, ICS; Teruel, MS, 2021)		3.51
"Methylmalonic acid (MMA) is a sensitive indicator of B12 status."( South Asian Ethnicity Is Related to the Highest Risk of Vitamin B12 Deficiency in Pregnant Canadian Women.
Green, TJ; Isman, C; Jeruszka-Bielak, M; Lamers, Y; Li, W; Schroder, TH, 2017)		1.18
"Methylmalonic acidemia (MMA) is a common organic acidemia, mainly due to methylmalonyl-CoA mutase (MCM) or its coenzyme cobalamin (VitB12) metabolic disorders. "( Atypical hemolytic uremic syndrome induced by CblC subtype of methylmalonic academia: A case report and literature review.
Chen, M; Wang, D; Yang, J; Yang, Q; Zhuang, J, 2017)		1.9
"Methylmalonic aciduria (MMA) is a disorder of organic acid metabolism resulting from a functional defect of the mitochondrial enzyme, methylmalonyl-CoA mutase (MCM). "( TAT-MTS-MCM fusion proteins reduce MMA levels and improve mitochondrial activity and liver function in MCM-deficient cells.
Erlich-Hadad, T; Feldman, A; Greif, H; Hadad, R; Lictenstein, M; Lorberboum-Galski, H, 2018)		1.92
"Methylmalonic acidemia (MMA) is an autosomal recessive disease of organic acidemia."( Management of adult-onset methylmalonic acidemia with hypotonia and acute respiratory failure: A case report.
Chang, HT; Chang, MY; Chu, CC; Hsu, YL; Zhao, Z, 2018)		2.22
"Methylmalonic aciduria (MMAuria) is an inborn error of metabolism leading to neurological deterioration. "( New in vitro model derived from brain-specific Mut-/- mice confirms cerebral ammonium accumulation in methylmalonic aciduria.
Ballhausen, D; Baumgartner, M; Braissant, O; Cudré-Cung, HP; do Vale-Pereira, S; Forny, P; Gallart-Ayala, H; Gonzalez-Melo, M; Henry, H; Remacle, N; Teav, T, 2018)		2.14
"The methylmalonic acidemia is an inborn error of metabolism (IEM) characterized by methylmalonic acid (MMA) accumulation in body fluids and tissues, causing neurological dysfunction, mitochondrial failure and oxidative stress. "( Chronic administration of methylmalonate on young rats alters neuroinflammatory markers and spatial memory.
Bobinski, F; da Silva Fiorin, F; de Oliveira Ferreira, AP; de Oliveira, CV; Della-Pace, ID; Dos Santos, AR; Duarte, MM; Fighera, MR; Funck, VR; Furian, AF; Nogueira, CW; Oliveira, MS; Pinton, S; Ribeiro, LR; Royes, LF, 2013)		0.95
"Methylmalonic acidemia (MMA) is a metabolic disorder, which is caused by a deficiency of the mitochondrial enzyme methylmalonyl-CoA mutase. "( A Primary Study on Down-Regulated miR-9-1 and Its Biological Significances in Methylmalonic Acidemia.
Duan, R; Gao, H; Guan, W; Jia, Y; Li, Y; Peng, T; Teng, J; Wang, X, 2014)		2.07
"Methylmalonic aciduria is a rare disorder of organic acid metabolism with limited therapeutic options, resulting in high morbidity and mortality. "( Correction of methylmalonic aciduria in vivo using a codon-optimized lentiviral vector.
Anson, DS; Fletcher, JM; McIntyre, C; Peters, HL; Ranieri, E; Wong, ES, 2014)		2.21
"Methylmalonic acidemia (MMA) is an autosomal-recessive inborn metabolic disorder that results from a deficiency in methylmalonyl-coenzyme A mutase or its cofactor, adenosylcobalamin. "( MicroRNA-9 regulates neural apoptosis in methylmalonic acidemia via targeting BCL2L11.
Duan, R; Gao, H; Guan, W; Jia, Y; Li, L; Li, Y; Lu, J; Peng, T; Teng, J; Wang, X, 2014)		2.11
"Methylmalonic acid (MMA) is a sensitive and specific functional biomarker of vitamin B-12 status, commonly assessed in plasma or serum. "( Methylmalonic acid quantified in dried blood spots provides a precise, valid, and stable measure of functional vitamin B-12 status in healthy women.
Lamers, Y; Quay, TA; Schroder, TH, 2014)		3.29
"Methylmalonic Acidemia (MMA) is an inborn error of metabolism that results in accumulation of methylmalonic acid in blood and increased excretion in urine. "( Methylmalonic Acidemia.
Ali, S; Awais Ul Hassan Shah, S; Mahmud, S, 2015)		3.3
"Methylmalonic acid (MMA) is a by-product of propionic acid metabolism through the vitamin B12 (cobalamin)-dependent enzyme methylmalonyl CoA mutase. "( A Common Polymorphism in HIBCH Influences Methylmalonic Acid Concentrations in Blood Independently of Cobalamin.
Abaan, HO; Bailey-Wilson, JE; Brody, LC; Casey, M; Cropp, CD; Cunningham, C; Kim, Y; McGaughey, DM; McNulty, H; Mills, JL; Molloy, AM; O'Neill, MB; Pangilinan, F; Shane, B; Strain, JJ; Ueland, PM; Velkova, A; Ward, M; Wilson, AF, 2016)		2.14
"Methylmalonic acidemia (MMA) is an autosomal-recessive inborn error of metabolism."( Methylmalonic acidemia: brain imaging findings in 52 children and a review of the literature.
Ghanaati, H; Molaei, S; Radmanesh, A; Robertson, RL; Zaman, T; Zamani, AA, 2008)		3.23
"Methylmalonic acidemia (MMA) is a heterogeneous inborn error of propionate metabolism. "( Delay of renal progression in methylmalonic acidemia using angiotensin II inhibition: a case report.
Ha, TS; Hong, EJ; Lee, JS, )		1.86
"Methylmalonic acidemia (MMA) is a multifactorial autosomal recessive inborn error of organic acid metabolism, often presenting with neurological symptoms. "( Fractional anisotropy for assessment of white matter tracts injury in methylmalonic acidemia.
Gao, Y; Guan, WY; Han, LS; Li, YH; Wang, J; Zhang, YZ, 2009)		2.03
"Methylmalonic acidemia (MMA) is an organic acidemia caused by deficient activity of the mitochondrial enzyme methylmalonyl-CoA mutase (MUT). "( Long-term rescue of a lethal murine model of methylmalonic acidemia using adeno-associated viral gene therapy.
Chandler, RJ; Venditti, CP, 2010)		2.06
"Methylmalonic acidemia is a lethal inborn error of metabolism that causes mitochondrial impairment, multi-organ dysfunction and a shortened lifespan. "( Ion-abrasion scanning electron microscopy reveals distorted liver mitochondrial morphology in murine methylmalonic acidemia.
Chandler, RJ; Lowekamp, BC; Murphy, GE; Narasimha, R; Subramaniam, S; Venditti, CP; Zerfas, PM, 2010)		2.02
"Methylmalonic aciduria is a common inherited metabolic disorder. "( Prenatal diagnosis of methylmalonic aciduria by measuring methylmalonic acid in dried amniotic fluid on filter paper using gas chromatography-mass spectrometry.
Inoue, Y; Ohse, M, 2011)		2.13
"Methylmalonic acid (MMA) is a metabolic intermediate transformed to succinic acid (SA) by a vitamin B(12)-dependent catalytic step, and is broadly used as a clinical biomarker of functional vitamin B12 status. "( Methylmalonic acid quantification in low serum volumes by UPLC-MS/MS.
Allen, LH; Keyes, WR; Newman, JW; Pedersen, TL; Shahab-Ferdows, S, 2011)		3.25
"Methylmalonic aciduria is an inborn error of metabolism that causes renal failure and tubulointerstitial (TI) nephritis as complications. "( Expression of proinflammatory factors in renal cortex induced by methylmalonic acid.
Aldámiz-Echevarría, L; Andrade, F; de Las Heras, J; Goyenechea, E; Lage, S; Prieto, JÁ; Ruiz, N, 2012)		2.06
"Methylmalonic acid (MMA) is a functional biomarker of vitamin B12 deficiency. "( Highly sensitive and selective measurement of underivatized methylmalonic acid in serum and plasma by liquid chromatography-tandem mass spectrometry.
El-Khoury, JM; Gabler, J; Spatholt, R; Wang, S; Yuan, C, 2012)		2.06
"Methylmalonic acidurias are a heterogeneous group of inborn errors of branched-chain amino acid metabolism. "( Renal dysfunction in methylmalonic acidurias: review for the pediatric nephrologist.
Hörster, F; Morath, MA; Sauer, SW, 2013)		2.15
"Methylmalonic aciduria is an inborn error of metabolism characterized by accumulation of methylmalonate (MMA), propionate and 2-methylcitrate (2-MCA) in body fluids. "( Brain damage in methylmalonic aciduria: 2-methylcitrate induces cerebral ammonium accumulation and apoptosis in 3D organotypic brain cell cultures.
Ballhausen, D; Bonafé, L; Braissant, O; Henry, H; Jafari, P; Zavadakova, P, 2013)		2.18
"Methylmalonic acidemia (MMA) is an autosomal recessive disease of branched chain amino acid metabolism. "( Living-related liver transplantation for methylmalonic acidemia: report of one case.
Chen, HL; Chien, YH; Chu, SY; Ho, MJ; Hsui, JY; Hwu, WL; Lee, PH; Lu, FL, )		1.84
"Methylmalonic aciduria is a human autosomal recessive disorder of organic acid metabolism resulting from a functional defect in the activity of the enzyme methylmalonyl-CoA mutase. "( A knock-out mouse model for methylmalonic aciduria resulting in neonatal lethality.
Fowler, KJ; Gazeas, S; Ioannou, PA; Kahler, SG; Nefedov, M; Peters, H; Pitt, J; Sarsero, J, 2003)		2.06
"Methylmalonic acidemia (MMA) is a multifactorial autosomal recessive inborn error of organic acid metabolism, often presenting with neurologic findings. "( Imaging of the brain, including diffusion-weighted imaging in methylmalonic acidemia.
Given, CA; Michel, SJ; Robertson, WC, 2004)		2.01
"Methylmalonic acidemia (MMA) is an inborn disorder of amino acid metabolism, often presenting with neurological symptoms. "( Magnetic resonance imaging and diffusion-weighted imaging in methylmalonic acidemia.
Ayata, A; Baykal, B; Koroglu, M; Oktem, F; Oral, B; Oyar, O; Yeşildağ, A; Yildiz, H, 2005)		2.01
"Methylmalonic acidaemia (MMA) is a rare autosomal recessive inborn error of metabolism that typically presents in infancy with recurrent episodes of metabolic acidosis, developmental delay and failure to thrive. "( Management of methylmalonic acidaemia by combined liver-kidney transplantation.
Enns, GM; Millan, MT; Nagarajan, S; Sarwal, MM; Winter, S, 2005)		2.13
"Methylmalonic acidemia is an inherited metabolic disorder biochemically characterized by tissue accumulation of methylmalonic acid (MMA) and clinically by progressive neurological deterioration and kidney failure, whose pathophysiology is so far poorly established. "( Differential inhibitory effects of methylmalonic acid on respiratory chain complex activities in rat tissues.
Dutra-Filho, CS; Ferreira, Gda C; Pettenuzzo, LF; Schmidt, AL; Wajner, M; Wyse, AT, 2006)		2.05
"Methylmalonic acidemia is a rare disease, and its neuropathology in childhood has been rarely reported. "( Neuropathology of methylmalonic acidemia in a child.
Hirose, S; Iwasaki, H; Kanaumi, T; Kodama, T; Takashima, S, 2006)		2.11
"Methylmalonic acid (MMA) is a biochemical marker for cobalamin deficiency, particularly in cases where the cobalamin concentration is moderately decreased or in the low-normal range. "( Analysis of methylmalonic acid in plasma by liquid chromatography-tandem mass spectrometry.
Brandslund, I; Schmedes, A, 2006)		2.16
"Methylmalonic acidemia is an inherited metabolic disorder that leads to brain damage associated to the accumulation of methylmalonic acid (MMA) and impairment of energy metabolism. "( Diazoxide protects against methylmalonate-induced neuronal toxicity.
Castilho, RF; Fornazari, M; Kowaltowski, AJ; Maciel, EN, 2006)		1.78
"Methylmalonic acidemia (MMAemia) is an inherited metabolic disorder of branched amino acid and odd-chain fatty acid metabolism, involving a defect in the conversion of methylmalonyl-coenzyme A to succinyl-coenzyme A. "( Lactate dehydrogenase activity is inhibited by methylmalonate in vitro.
Castilho, RF; Maciel, EN; Mirandola, SR; Saad, LO, 2006)		1.78
"Methylmalonic aciduria is a common organic aciduria disease. "( Clinical and biochemical studies on Chinese patients with methylmalonic aciduria.
Hasegawa, Y; Jiang, Y; Qin, J; Song, J; Sun, F; Wu, X; Yamaguchi, S; Yang, Y; Zhang, Y, 2006)		2.02
"Methylmalonic acidemia (MMA) is an inborn error of organic acid metabolism. "( Renal transplant in methylmalonic acidemia: could it be the best option? Report on a case at 10 years and review of the literature.
Barsotti, P; Berloco, P; Carducci, C; Elli, M; Lubrano, R; Raggi, C; Rossi, M; Travasso, E, 2007)		2.11
"The methylmalonic aciduria is an organic acidemia, inherited as autosomic recessive trait, caused by a deficiency of the methylmalonyl-CoA mutase, or by defects in the biosynthesis of the cofactor adenosylcobalamin. "( [Diagnosis and treatment of methylmalonic aciduria: a case report].
Domínguez, CL; Mahfoud, A; Merinero, B; Pérez, A; Pérez, B; Rizzo, C, 2007)		1.19
"Methylmalonic acid (MMA) is an endogenous convulsing compound that accumulates in methylmalonic acidemia, an inborn error of the metabolism characterized by severe neurological dysfunction, including seizures. "( Convulsions induced by methylmalonic acid are associated with glutamic acid decarboxylase inhibition in rats: a role for GABA in the seizures presented by methylmalonic acidemic patients?
Fighera, MR; Garrido-Sanabria, ER; Malfatti, CR; Mello, CF; Muller, AP; Paquetti, L; Perry, ML; Rigo, FK; Schweigert, ID, 2007)		2.09
"Methylmalonic acidaemia (MMA) is a heterogeneous group of rare genetic metabolic disorders caused by defects related to intracellular cobalamin (vitamin B(12)) metabolism. "( Methylmalonic acidaemia leads to increased production of reactive oxygen species and induction of apoptosis through the mitochondrial/caspase pathway.
Alvarez-Barrientos, A; Desviat, LR; Pérez, B; Richard, E; Ugarte, M, 2007)		3.23
"Methylmalonic aciduria (MMA) is a common one of the congenital disorders of organic acids metabolism. "( [Clinical studies on fifty-seven Chinese patients with combined methylmalonic aciduria and homocysteinemia].
Dong, JH; Jiang, YW; Liu, P; Qin, J; Song, JQ; Wang, LF; Wu, XR; Yan, R; Yang, YL; Zhang, Y; Zhang, YH, 2007)		2.02
"Methylmalonic acidaemia (MMA) is a genetic disorder caused by defects in methylmalonyl-CoA mutase or in any of the different proteins involved in the synthesis of adenosylcobalamin. "( Methylmalonic acidaemia: examination of genotype and biochemical data in 32 patients belonging to mut, cblA or cblB complementation group.
Aldamiz-Echevarría, L; Campos, J; Cornejo, V; Del Toro, M; Desviat, LR; García, MJ; Mahfoud, A; Martínez, MA; Martínez-Pardo, M; Merinero, B; Parini, R; Pedrón, C; Peña-Quintana, L; Pérez, B; Pérez, M; Pérez-Cerdá, C; Pourfarzam, M; Rincón, A; Sala, PR; Ugarte, M, 2008)		3.23
"Methylmalonic acidemia is a recessively inherited inborn error of metabolism presenting with metabolic acidosis, vomiting, lethargy, anorexia, and hypotonia. "( [Methylmalonic acidemia: classification, diagnosis and therapy].
Cantani, A, )		2.48
"Methylmalonic aciduria is a rare metabolic disorder of amino acid metabolism that is characterized by accumulation of large amounts of methylmalonic acid in the blood and urine. "( Methylmalonic aciduria in pregnancy: a case report.
Diss, E; Iams, J; Reed, N; Roe, C; Roe, DS, 1995)		3.18
"Methylmalonic acidaemia is an inherited metabolic disorder caused by a severe deficiency of the activity of the enzyme L-methylmalonyl-CoA mutase or its cofactor 5'-deoxyadenosylcobalamin, resulting in tissue accumulation of large quantities of methylmalonic acid. "( Neurological dysfunction in methylmalonic acidaemia is probably related to the inhibitory effect of methylmalonate on brain energy production.
Coelho, JC; Wajner, M, 1997)		2.03
"Methylmalonic aciduria (MMA) is an autosomal recessive inborn error of metabolism that results from functional defects in methylmalonyl CoA mutase (MCM), a nuclear-encoded, mitochondrial enzyme that uses the vitamin B12 derivative, adenosylcobalamin (AdoCbl) as a cofactor. "( Seven novel mutations in mut methylmalonic aciduria.
Adjalla, CE; Chan, A; Evans, S; Gilfix, BM; Hosack, AR; Lamothe, E; Matiaszuk, NV; Rosenblatt, DS; Sun, S, 1998)		2.03
"Methylmalonic acidaemia is an inborn error of metabolism characterized by recurrent episodes of life-threatening ketoacidosis. "( Successful pregnancy in severe methylmalonic acidaemia.
Desnick, RJ; Eddleman, K; Gaddipati, S; Sansaricq, C; Snyderman, SE; Wasserstein, MP, 1999)		2.03
"Methylmalonic acidemia is an inborn error of metabolism known to be a cause of ketoacidosis and mental retardation. "( High cognitive outcome in an adolescent with mut- methylmalonic acidemia.
Levy, HL; Repetto, GM; Varvogli, L; Waisbren, SE, 2000)		2
"Methylmalonic aciduria (MMA) is an autosomal-recessive disorder caused by inadequate function of methylmalonyl-CoA mutase (MCM), a nuclear-encoded, mitochondrial enzyme that uses adenosylcobalamin as a cofactor. "( mut0 methylmalonic acidemia: eleven novel mutations of the methylmalonyl CoA mutase including a deletion-insertion mutation.
Baumgartner, ER; Fuchshuber, A; Hildebrandt, F; Mucha, B; Vollmer, M, 2000)		2.26
"Methylmalonic acidemia is an inborn disorder of amino acid metabolism that commonly presents with neurologic deficits. "( Multi-slice proton MR spectroscopy and diffusion-weighted imaging in methylmalonic acidemia: report of two cases and review of the literature.
Barker, PB; Melhem, ER; Trinh, BC, 2001)		1.99
"Methylmalonic acidemia is an inborn error of organic acid metabolism resulting from defects in methylmalonyl CoA mutase. "( Plasma amino acid and urine organic acid analyses of methylmalonic acidemia in a Thai infant.
Chokchaichamnankit, D; Liammongkolkul, S; Srisomsap', C; Svasti, J; Wasant, P, 1999)		2
"Methylmalonic acid (MMA) is a dicarboxylic acid whose concentration can be increased in blood and urine in patients with an inborn error of metabolism or vitamin B(12) deficiency. "( Analysis of dicarboxylic acids by tandem mass spectrometry. High-throughput quantitative measurement of methylmalonic acid in serum, plasma, and urine.
Komaromy-Hiller, G; Kushnir, MM; Roberts, WL; Shushan, B; Urry, FM, 2001)		1.97
"Methylmalonic acidemia (MMA) is an inborn error of organic acid metabolism that occurs in infancy with hypotonia, vomiting, dehydration, lethargy and failure to thrive and is biochemically characterized by metabolic ketoacidosis, hyperammonemia and sometimes hyperglycinemia. "( Kidney transplantation in a girl with methylmalonic acidemia and end stage renal failure.
Barsotti, P; Castello, MA; Cristaldi, S; Lubrano, R; Scateni, S; Scoppi, P; Travasso, E, 2001)		2.02
"Methylmalonic acidemia is a heterogeneous inborn error of propionate metabolism. "( Progressive renal insufficiency in methylmalonic acidemia.
Friedman, AL; Molteni, KH; Oberley, TD; Wolff, JA, 1991)		2
"Methylmalonic acidemia is an organic acidemia caused by deficient activity of L-methylmalonyl-CoA mutase or its cofactor cyanocobalamin and it is biochemically characterized by an accumulation of methylmalonic acid (MMA) in tissue and body fluids of patients. "( Methylmalonic Acid Impairs Cell Respiration and Glutamate Uptake in C6 Rat Glioma Cells: Implications for Methylmalonic Acidemia.
Alberto-Silva, C; Carrettiero, DC; Costa, RT; Ribeiro, CAJ; Santos, MB, 2023)		3.8
"Methylmalonic acidemia (MMA) is a rare and severe inherited metabolic disease typically caused by mutations of the methylmalonyl-CoA mutase (MMUT) gene. "( Growth advantage of corrected hepatocytes in a juvenile model of methylmalonic acidemia following liver directed adeno-associated viral mediated nuclease-free genome editing.
Chandler, RJ; Chau, N; Gordo, S; Hoffmann, V; Liao, J; Ramesh, N; Venditti, CP; Venturoni, LE, )		1.81
"Methylmalonic acidemia is a neurometabolic disorder biochemically characterized by the accumulation of methylmalonic acid (MMA) in different tissues, including the central nervous system (CNS). "( Methylmalonic acid induces inflammatory response and redox homeostasis disruption in C6 astroglial cells: potential glioprotective roles of melatonin and resveratrol.
Bobermin, LD; de Souza Almeida, RR; Gonçalves, CA; Leipnitz, G; Parmeggiani, B; Quincozes-Santos, A; Souza, DO; Wajner, M; Wartchow, KM, 2022)		3.61
"Methylmalonic acidemia (MMA) is a severe inborn error of metabolism that is characterized by pleiotropic metabolic perturbations and multiorgan pathology. "( New insights into the pathophysiology of methylmalonic acidemia.
Head, PE; Meier, JL; Venditti, CP, 2023)		2.62
"Methylmalonic Acidemia (MMA) is a heterogenous group of inborn errors of metabolism caused by a defect in the methylmalonyl-CoA mutase (MMUT) enzyme or the synthesis and transport of its cofactor, 5'-deoxy-adenosylcobalamin. "( Biomarkers to predict disease progression and therapeutic response in isolated methylmalonic acidemia.
Catesini, G; Dionisi-Vici, C; Ferry, S; Gagné, J; Gebremariam, A; Hall, C; Manoli, I; Martinelli, D; McCoy, S; Pass, AR; Rizzo, C; Sacchetti, E; Sloan, JL; Spada, M; Van Ryzin, C; Venditti, CP, 2023)		2.58
"Methylmalonic Acidurias (MMAs) are a group of inborn errors of metabolism (IEMs), specifically of propionate catabolism characterized by gastrointestinal and neurometabolic manifestations resulting from a deficiency in the function of methylmalonyl-CoA mutase, methylmalonyl-CoA epimerase, and cobalamin metabolism. "( A False-Positive Case of Methylmalonic Aciduria by Tandem Mass Spectrometry Newborn Screening Dependent on Maternal Malnutrition in Pregnancy.
Brindisino, P; Cicalini, I; Consalvo, A; De Laurenzi, V; Dionisi-Vici, C; Gasparroni, G; Gazzolo, D; Pieragostino, D; Rizzo, C; Rossi, C; Semeraro, D; Valentinuzzi, S; Zucchelli, M, 2020)		2.3
"Methylmalonic acidemia is an inborn metabolic disease of propionate catabolism, biochemically characterized by accumulation of methylmalonic acid (MMA) to millimolar concentrations in tissues and body fluids. "( The Effect of Methylmalonic Acid Treatment on Human Neuronal Cell Coenzyme Q
Bennett, E; Boan, EJ; Hargreaves, IP; Heaton, RA; Philips, S; Proctor, EC; Turton, N, 2020)		2.36
"Methylmalonic acidemia is an inherited organic acid metabolic disease. "( A rare mutation c.1663G > A (p.A555T) in the MMUT gene associated with mild clinical and biochemical phenotypes of methylmalonic acidemia in 30 Chinese patients.
Chen, X; Chen, Y; Feng, J; Gong, Z; Gu, X; Han, L; Hu, H; Ji, W; Liang, L; Lu, D; Niu, T; Qiu, W; Shen, L; Shuai, R; Wei, H; Wu, S; Xu, P; Yang, C; Ye, J; Yu, Y; Zhan, X; Zhang, H; Zou, H, 2021)		2.27
"Methylmalonic acidemia is a rare metabolic disorder caused by the deficient activity of l-methylmalonyl-CoA mutase or its cofactor 5-deoxyadenosylcobalamin and is characterized by accumulation of methylmalonic acid (MMA) and alternative metabolites. "( Methylmalonic Acid Compromises Respiration and Reduces the Expression of Differentiation Markers of SH-SY5Y Human Neuroblastoma Cells.
Carrettiero, DC; da Costa, RT; de Almeida, RP; Dos Santos, MB; Ribeiro, CAJ; Silva, ICS; Teruel, MS, 2021)		3.51
"Methylmalonic acid (MMA) is a sensitive indicator of B12 status."( South Asian Ethnicity Is Related to the Highest Risk of Vitamin B12 Deficiency in Pregnant Canadian Women.
Green, TJ; Isman, C; Jeruszka-Bielak, M; Lamers, Y; Li, W; Schroder, TH, 2017)		1.18
"Methylmalonic acidemia (MMA) is a common organic acidemia, mainly due to methylmalonyl-CoA mutase (MCM) or its coenzyme cobalamin (VitB12) metabolic disorders. "( Atypical hemolytic uremic syndrome induced by CblC subtype of methylmalonic academia: A case report and literature review.
Chen, M; Wang, D; Yang, J; Yang, Q; Zhuang, J, 2017)		1.9
"Methylmalonic aciduria (MMA) is a disorder of organic acid metabolism resulting from a functional defect of the mitochondrial enzyme, methylmalonyl-CoA mutase (MCM). "( TAT-MTS-MCM fusion proteins reduce MMA levels and improve mitochondrial activity and liver function in MCM-deficient cells.
Erlich-Hadad, T; Feldman, A; Greif, H; Hadad, R; Lictenstein, M; Lorberboum-Galski, H, 2018)		1.92
"Methylmalonic acidemia (MMA) is an autosomal recessive disease of organic acidemia."( Management of adult-onset methylmalonic acidemia with hypotonia and acute respiratory failure: A case report.
Chang, HT; Chang, MY; Chu, CC; Hsu, YL; Zhao, Z, 2018)		2.22
"Methylmalonic aciduria (MMAuria) is an inborn error of metabolism leading to neurological deterioration. "( New in vitro model derived from brain-specific Mut-/- mice confirms cerebral ammonium accumulation in methylmalonic aciduria.
Ballhausen, D; Baumgartner, M; Braissant, O; Cudré-Cung, HP; do Vale-Pereira, S; Forny, P; Gallart-Ayala, H; Gonzalez-Melo, M; Henry, H; Remacle, N; Teav, T, 2018)		2.14
"The methylmalonic acidemia is an inborn error of metabolism (IEM) characterized by methylmalonic acid (MMA) accumulation in body fluids and tissues, causing neurological dysfunction, mitochondrial failure and oxidative stress. "( Chronic administration of methylmalonate on young rats alters neuroinflammatory markers and spatial memory.
Bobinski, F; da Silva Fiorin, F; de Oliveira Ferreira, AP; de Oliveira, CV; Della-Pace, ID; Dos Santos, AR; Duarte, MM; Fighera, MR; Funck, VR; Furian, AF; Nogueira, CW; Oliveira, MS; Pinton, S; Ribeiro, LR; Royes, LF, 2013)		0.95
"Methylmalonic acidemia (MMA) is a metabolic disorder, which is caused by a deficiency of the mitochondrial enzyme methylmalonyl-CoA mutase. "( A Primary Study on Down-Regulated miR-9-1 and Its Biological Significances in Methylmalonic Acidemia.
Duan, R; Gao, H; Guan, W; Jia, Y; Li, Y; Peng, T; Teng, J; Wang, X, 2014)		2.07
"Methylmalonic aciduria is a rare disorder of organic acid metabolism with limited therapeutic options, resulting in high morbidity and mortality. "( Correction of methylmalonic aciduria in vivo using a codon-optimized lentiviral vector.
Anson, DS; Fletcher, JM; McIntyre, C; Peters, HL; Ranieri, E; Wong, ES, 2014)		2.21
"Methylmalonic acidemia (MMA) is an autosomal-recessive inborn metabolic disorder that results from a deficiency in methylmalonyl-coenzyme A mutase or its cofactor, adenosylcobalamin. "( MicroRNA-9 regulates neural apoptosis in methylmalonic acidemia via targeting BCL2L11.
Duan, R; Gao, H; Guan, W; Jia, Y; Li, L; Li, Y; Lu, J; Peng, T; Teng, J; Wang, X, 2014)		2.11
"Methylmalonic acid (MMA) is a sensitive and specific functional biomarker of vitamin B-12 status, commonly assessed in plasma or serum. "( Methylmalonic acid quantified in dried blood spots provides a precise, valid, and stable measure of functional vitamin B-12 status in healthy women.
Lamers, Y; Quay, TA; Schroder, TH, 2014)		3.29
"Methylmalonic Acidemia (MMA) is an inborn error of metabolism that results in accumulation of methylmalonic acid in blood and increased excretion in urine. "( Methylmalonic Acidemia.
Ali, S; Awais Ul Hassan Shah, S; Mahmud, S, 2015)		3.3
"Methylmalonic acid (MMA) is a by-product of propionic acid metabolism through the vitamin B12 (cobalamin)-dependent enzyme methylmalonyl CoA mutase. "( A Common Polymorphism in HIBCH Influences Methylmalonic Acid Concentrations in Blood Independently of Cobalamin.
Abaan, HO; Bailey-Wilson, JE; Brody, LC; Casey, M; Cropp, CD; Cunningham, C; Kim, Y; McGaughey, DM; McNulty, H; Mills, JL; Molloy, AM; O'Neill, MB; Pangilinan, F; Shane, B; Strain, JJ; Ueland, PM; Velkova, A; Ward, M; Wilson, AF, 2016)		2.14
"Methylmalonic acidemia (MMA) is an autosomal-recessive inborn error of metabolism."( Methylmalonic acidemia: brain imaging findings in 52 children and a review of the literature.
Ghanaati, H; Molaei, S; Radmanesh, A; Robertson, RL; Zaman, T; Zamani, AA, 2008)		3.23
"Methylmalonic acidemia (MMA) is a heterogeneous inborn error of propionate metabolism. "( Delay of renal progression in methylmalonic acidemia using angiotensin II inhibition: a case report.
Ha, TS; Hong, EJ; Lee, JS, )		1.86
"Methylmalonic acidemia (MMA) is a multifactorial autosomal recessive inborn error of organic acid metabolism, often presenting with neurological symptoms. "( Fractional anisotropy for assessment of white matter tracts injury in methylmalonic acidemia.
Gao, Y; Guan, WY; Han, LS; Li, YH; Wang, J; Zhang, YZ, 2009)		2.03
"Methylmalonic acidemia (MMA) is an organic acidemia caused by deficient activity of the mitochondrial enzyme methylmalonyl-CoA mutase (MUT). "( Long-term rescue of a lethal murine model of methylmalonic acidemia using adeno-associated viral gene therapy.
Chandler, RJ; Venditti, CP, 2010)		2.06
"Methylmalonic acidemia is a lethal inborn error of metabolism that causes mitochondrial impairment, multi-organ dysfunction and a shortened lifespan. "( Ion-abrasion scanning electron microscopy reveals distorted liver mitochondrial morphology in murine methylmalonic acidemia.
Chandler, RJ; Lowekamp, BC; Murphy, GE; Narasimha, R; Subramaniam, S; Venditti, CP; Zerfas, PM, 2010)		2.02
"Methylmalonic aciduria is a common inherited metabolic disorder. "( Prenatal diagnosis of methylmalonic aciduria by measuring methylmalonic acid in dried amniotic fluid on filter paper using gas chromatography-mass spectrometry.
Inoue, Y; Ohse, M, 2011)		2.13
"Methylmalonic acid (MMA) is a metabolic intermediate transformed to succinic acid (SA) by a vitamin B(12)-dependent catalytic step, and is broadly used as a clinical biomarker of functional vitamin B12 status. "( Methylmalonic acid quantification in low serum volumes by UPLC-MS/MS.
Allen, LH; Keyes, WR; Newman, JW; Pedersen, TL; Shahab-Ferdows, S, 2011)		3.25
"Methylmalonic aciduria is an inborn error of metabolism that causes renal failure and tubulointerstitial (TI) nephritis as complications. "( Expression of proinflammatory factors in renal cortex induced by methylmalonic acid.
Aldámiz-Echevarría, L; Andrade, F; de Las Heras, J; Goyenechea, E; Lage, S; Prieto, JÁ; Ruiz, N, 2012)		2.06
"Methylmalonic acid (MMA) is a functional biomarker of vitamin B12 deficiency. "( Highly sensitive and selective measurement of underivatized methylmalonic acid in serum and plasma by liquid chromatography-tandem mass spectrometry.
El-Khoury, JM; Gabler, J; Spatholt, R; Wang, S; Yuan, C, 2012)		2.06
"Methylmalonic acidurias are a heterogeneous group of inborn errors of branched-chain amino acid metabolism. "( Renal dysfunction in methylmalonic acidurias: review for the pediatric nephrologist.
Hörster, F; Morath, MA; Sauer, SW, 2013)		2.15
"Methylmalonic aciduria is an inborn error of metabolism characterized by accumulation of methylmalonate (MMA), propionate and 2-methylcitrate (2-MCA) in body fluids. "( Brain damage in methylmalonic aciduria: 2-methylcitrate induces cerebral ammonium accumulation and apoptosis in 3D organotypic brain cell cultures.
Ballhausen, D; Bonafé, L; Braissant, O; Henry, H; Jafari, P; Zavadakova, P, 2013)		2.18
"Methylmalonic acidemia (MMA) is an autosomal recessive disease of branched chain amino acid metabolism. "( Living-related liver transplantation for methylmalonic acidemia: report of one case.
Chen, HL; Chien, YH; Chu, SY; Ho, MJ; Hsui, JY; Hwu, WL; Lee, PH; Lu, FL, )		1.84
"Methylmalonic aciduria is a human autosomal recessive disorder of organic acid metabolism resulting from a functional defect in the activity of the enzyme methylmalonyl-CoA mutase. "( A knock-out mouse model for methylmalonic aciduria resulting in neonatal lethality.
Fowler, KJ; Gazeas, S; Ioannou, PA; Kahler, SG; Nefedov, M; Peters, H; Pitt, J; Sarsero, J, 2003)		2.06
"Methylmalonic acidemia (MMA) is a multifactorial autosomal recessive inborn error of organic acid metabolism, often presenting with neurologic findings. "( Imaging of the brain, including diffusion-weighted imaging in methylmalonic acidemia.
Given, CA; Michel, SJ; Robertson, WC, 2004)		2.01
"Methylmalonic acidemia (MMA) is an inborn disorder of amino acid metabolism, often presenting with neurological symptoms. "( Magnetic resonance imaging and diffusion-weighted imaging in methylmalonic acidemia.
Ayata, A; Baykal, B; Koroglu, M; Oktem, F; Oral, B; Oyar, O; Yeşildağ, A; Yildiz, H, 2005)		2.01
"Methylmalonic acidaemia (MMA) is a rare autosomal recessive inborn error of metabolism that typically presents in infancy with recurrent episodes of metabolic acidosis, developmental delay and failure to thrive. "( Management of methylmalonic acidaemia by combined liver-kidney transplantation.
Enns, GM; Millan, MT; Nagarajan, S; Sarwal, MM; Winter, S, 2005)		2.13
"Methylmalonic acidemia is an inherited metabolic disorder biochemically characterized by tissue accumulation of methylmalonic acid (MMA) and clinically by progressive neurological deterioration and kidney failure, whose pathophysiology is so far poorly established. "( Differential inhibitory effects of methylmalonic acid on respiratory chain complex activities in rat tissues.
Dutra-Filho, CS; Ferreira, Gda C; Pettenuzzo, LF; Schmidt, AL; Wajner, M; Wyse, AT, 2006)		2.05
"Methylmalonic acidemia is a rare disease, and its neuropathology in childhood has been rarely reported. "( Neuropathology of methylmalonic acidemia in a child.
Hirose, S; Iwasaki, H; Kanaumi, T; Kodama, T; Takashima, S, 2006)		2.11
"Methylmalonic acid (MMA) is a biochemical marker for cobalamin deficiency, particularly in cases where the cobalamin concentration is moderately decreased or in the low-normal range. "( Analysis of methylmalonic acid in plasma by liquid chromatography-tandem mass spectrometry.
Brandslund, I; Schmedes, A, 2006)		2.16
"Methylmalonic acidemia is an inherited metabolic disorder that leads to brain damage associated to the accumulation of methylmalonic acid (MMA) and impairment of energy metabolism. "( Diazoxide protects against methylmalonate-induced neuronal toxicity.
Castilho, RF; Fornazari, M; Kowaltowski, AJ; Maciel, EN, 2006)		1.78
"Methylmalonic acidemia (MMAemia) is an inherited metabolic disorder of branched amino acid and odd-chain fatty acid metabolism, involving a defect in the conversion of methylmalonyl-coenzyme A to succinyl-coenzyme A. "( Lactate dehydrogenase activity is inhibited by methylmalonate in vitro.
Castilho, RF; Maciel, EN; Mirandola, SR; Saad, LO, 2006)		1.78
"Methylmalonic aciduria is a common organic aciduria disease. "( Clinical and biochemical studies on Chinese patients with methylmalonic aciduria.
Hasegawa, Y; Jiang, Y; Qin, J; Song, J; Sun, F; Wu, X; Yamaguchi, S; Yang, Y; Zhang, Y, 2006)		2.02
"Methylmalonic acidemia (MMA) is an inborn error of organic acid metabolism. "( Renal transplant in methylmalonic acidemia: could it be the best option? Report on a case at 10 years and review of the literature.
Barsotti, P; Berloco, P; Carducci, C; Elli, M; Lubrano, R; Raggi, C; Rossi, M; Travasso, E, 2007)		2.11
"The methylmalonic aciduria is an organic acidemia, inherited as autosomic recessive trait, caused by a deficiency of the methylmalonyl-CoA mutase, or by defects in the biosynthesis of the cofactor adenosylcobalamin. "( [Diagnosis and treatment of methylmalonic aciduria: a case report].
Domínguez, CL; Mahfoud, A; Merinero, B; Pérez, A; Pérez, B; Rizzo, C, 2007)		1.19
"Methylmalonic acid (MMA) is an endogenous convulsing compound that accumulates in methylmalonic acidemia, an inborn error of the metabolism characterized by severe neurological dysfunction, including seizures. "( Convulsions induced by methylmalonic acid are associated with glutamic acid decarboxylase inhibition in rats: a role for GABA in the seizures presented by methylmalonic acidemic patients?
Fighera, MR; Garrido-Sanabria, ER; Malfatti, CR; Mello, CF; Muller, AP; Paquetti, L; Perry, ML; Rigo, FK; Schweigert, ID, 2007)		2.09
"Methylmalonic acidaemia (MMA) is a heterogeneous group of rare genetic metabolic disorders caused by defects related to intracellular cobalamin (vitamin B(12)) metabolism. "( Methylmalonic acidaemia leads to increased production of reactive oxygen species and induction of apoptosis through the mitochondrial/caspase pathway.
Alvarez-Barrientos, A; Desviat, LR; Pérez, B; Richard, E; Ugarte, M, 2007)		3.23
"Methylmalonic aciduria (MMA) is a common one of the congenital disorders of organic acids metabolism. "( [Clinical studies on fifty-seven Chinese patients with combined methylmalonic aciduria and homocysteinemia].
Dong, JH; Jiang, YW; Liu, P; Qin, J; Song, JQ; Wang, LF; Wu, XR; Yan, R; Yang, YL; Zhang, Y; Zhang, YH, 2007)		2.02
"Methylmalonic acidaemia (MMA) is a genetic disorder caused by defects in methylmalonyl-CoA mutase or in any of the different proteins involved in the synthesis of adenosylcobalamin. "( Methylmalonic acidaemia: examination of genotype and biochemical data in 32 patients belonging to mut, cblA or cblB complementation group.
Aldamiz-Echevarría, L; Campos, J; Cornejo, V; Del Toro, M; Desviat, LR; García, MJ; Mahfoud, A; Martínez, MA; Martínez-Pardo, M; Merinero, B; Parini, R; Pedrón, C; Peña-Quintana, L; Pérez, B; Pérez, M; Pérez-Cerdá, C; Pourfarzam, M; Rincón, A; Sala, PR; Ugarte, M, 2008)		3.23
"Methylmalonic acidemia is a recessively inherited inborn error of metabolism presenting with metabolic acidosis, vomiting, lethargy, anorexia, and hypotonia. "( [Methylmalonic acidemia: classification, diagnosis and therapy].
Cantani, A, )		2.48
"Methylmalonic aciduria is a rare metabolic disorder of amino acid metabolism that is characterized by accumulation of large amounts of methylmalonic acid in the blood and urine. "( Methylmalonic aciduria in pregnancy: a case report.
Diss, E; Iams, J; Reed, N; Roe, C; Roe, DS, 1995)		3.18
"Methylmalonic acidaemia is an inherited metabolic disorder caused by a severe deficiency of the activity of the enzyme L-methylmalonyl-CoA mutase or its cofactor 5'-deoxyadenosylcobalamin, resulting in tissue accumulation of large quantities of methylmalonic acid. "( Neurological dysfunction in methylmalonic acidaemia is probably related to the inhibitory effect of methylmalonate on brain energy production.
Coelho, JC; Wajner, M, 1997)		2.03
"Methylmalonic aciduria (MMA) is an autosomal recessive inborn error of metabolism that results from functional defects in methylmalonyl CoA mutase (MCM), a nuclear-encoded, mitochondrial enzyme that uses the vitamin B12 derivative, adenosylcobalamin (AdoCbl) as a cofactor. "( Seven novel mutations in mut methylmalonic aciduria.
Adjalla, CE; Chan, A; Evans, S; Gilfix, BM; Hosack, AR; Lamothe, E; Matiaszuk, NV; Rosenblatt, DS; Sun, S, 1998)		2.03
"Methylmalonic acidaemia is an inborn error of metabolism characterized by recurrent episodes of life-threatening ketoacidosis. "( Successful pregnancy in severe methylmalonic acidaemia.
Desnick, RJ; Eddleman, K; Gaddipati, S; Sansaricq, C; Snyderman, SE; Wasserstein, MP, 1999)		2.03
"Methylmalonic acidemia is an inborn error of metabolism known to be a cause of ketoacidosis and mental retardation. "( High cognitive outcome in an adolescent with mut- methylmalonic acidemia.
Levy, HL; Repetto, GM; Varvogli, L; Waisbren, SE, 2000)		2
"Methylmalonic aciduria (MMA) is an autosomal-recessive disorder caused by inadequate function of methylmalonyl-CoA mutase (MCM), a nuclear-encoded, mitochondrial enzyme that uses adenosylcobalamin as a cofactor. "( mut0 methylmalonic acidemia: eleven novel mutations of the methylmalonyl CoA mutase including a deletion-insertion mutation.
Baumgartner, ER; Fuchshuber, A; Hildebrandt, F; Mucha, B; Vollmer, M, 2000)		2.26
"Methylmalonic acidemia is an inborn disorder of amino acid metabolism that commonly presents with neurologic deficits. "( Multi-slice proton MR spectroscopy and diffusion-weighted imaging in methylmalonic acidemia: report of two cases and review of the literature.
Barker, PB; Melhem, ER; Trinh, BC, 2001)		1.99
"Methylmalonic acidemia is an inborn error of organic acid metabolism resulting from defects in methylmalonyl CoA mutase. "( Plasma amino acid and urine organic acid analyses of methylmalonic acidemia in a Thai infant.
Chokchaichamnankit, D; Liammongkolkul, S; Srisomsap', C; Svasti, J; Wasant, P, 1999)		2
"Methylmalonic acid (MMA) is a dicarboxylic acid whose concentration can be increased in blood and urine in patients with an inborn error of metabolism or vitamin B(12) deficiency. "( Analysis of dicarboxylic acids by tandem mass spectrometry. High-throughput quantitative measurement of methylmalonic acid in serum, plasma, and urine.
Komaromy-Hiller, G; Kushnir, MM; Roberts, WL; Shushan, B; Urry, FM, 2001)		1.97
"Methylmalonic acidemia (MMA) is an inborn error of organic acid metabolism that occurs in infancy with hypotonia, vomiting, dehydration, lethargy and failure to thrive and is biochemically characterized by metabolic ketoacidosis, hyperammonemia and sometimes hyperglycinemia. "( Kidney transplantation in a girl with methylmalonic acidemia and end stage renal failure.
Barsotti, P; Castello, MA; Cristaldi, S; Lubrano, R; Scateni, S; Scoppi, P; Travasso, E, 2001)		2.02
"Methylmalonic acidemia is a heterogeneous inborn error of propionate metabolism. "( Progressive renal insufficiency in methylmalonic acidemia.
Friedman, AL; Molteni, KH; Oberley, TD; Wolff, JA, 1991)		2

Effects

No methylmalonic acidemia has been reported in children with CDKL5 disorder.

ExcerptReferenceRelevance
"No methylmalonic acidemia has been reported in children with CDKL5 disorder."( A male case with CDKL5-associated encephalopathy manifesting transient methylmalonic acidemia.
Akamine, S; Fukai, R; Hara, T; Ishizaki, Y; Kimura, M; Koga, H; Matsumoto, N; Miyake, N; Ohga, S; Ohkubo, K; Saitsu, H; Sakai, Y; Sakamoto, O; Sakata, A; Sanefuji, M; Torisu, H; Yamaguchi, S, 2018)		1.23
"No methylmalonic acidemia has been reported in children with CDKL5 disorder."( A male case with CDKL5-associated encephalopathy manifesting transient methylmalonic acidemia.
Akamine, S; Fukai, R; Hara, T; Ishizaki, Y; Kimura, M; Koga, H; Matsumoto, N; Miyake, N; Ohga, S; Ohkubo, K; Saitsu, H; Sakai, Y; Sakamoto, O; Sakata, A; Sanefuji, M; Torisu, H; Yamaguchi, S, 2018)		1.23

Treatment

Methylmalonic aciduria is treated with a natural protein-restricted diet with adequate energy intake to sustain metabolic balance. Pretreatment methylmalonic Acid (MMA) levels significantly and independently predict grade 3/4 diarrhea and mucositis.

ExcerptReferenceRelevance
"Methylmalonic aciduria is treated with a natural protein-restricted diet with adequate energy intake to sustain metabolic balance. "( Dietary Treatment from Birth to Pregnancy in a Woman with Methylmalonic Aciduria.
Kowalik, A; MacDonald, A; Sykut-Cegielska, J, 2021)		2.31
"Pretreatment methylmalonic acid (MMA) levels significantly and independently predict grade 3/4 diarrhea and mucositis; however, these toxicities are still predicted by tHcy alone."( Homocysteine and methylmalonic acid: markers to predict and avoid toxicity from pemetrexed therapy.
Allen, RH; Baker, SD; Calvert, AH; Nelson, K; Niyikiza, C; Paoletti, P; Rusthoven, JJ; Seitz, DE; Stabler, SP; Walling, JM, 2002)		1.01
"Methylmalonic aciduria is treated with a natural protein-restricted diet with adequate energy intake to sustain metabolic balance. "( Dietary Treatment from Birth to Pregnancy in a Woman with Methylmalonic Aciduria.
Kowalik, A; MacDonald, A; Sykut-Cegielska, J, 2021)		2.31
"Pretreatment methylmalonic acid (MMA) levels significantly and independently predict grade 3/4 diarrhea and mucositis; however, these toxicities are still predicted by tHcy alone."( Homocysteine and methylmalonic acid: markers to predict and avoid toxicity from pemetrexed therapy.
Allen, RH; Baker, SD; Calvert, AH; Nelson, K; Niyikiza, C; Paoletti, P; Rusthoven, JJ; Seitz, DE; Stabler, SP; Walling, JM, 2002)		1.01

Toxicity

ExcerptReferenceRelevance
"Animals are known to convert inorganic selenium to less toxic methylated compounds such as dimethylselenide (DMSe) and trimethylselenonium (TMSe)."( Effect of vitamin B12 status on selenium methylation and toxicity in rats: in vivo and in vitro studies.
Chen, CL; Whanger, PD, 1993)		0.29
" The pathomechanisms involved are still unknown, a contribution of toxic organic acids, in particular MMA, has been suggested."( Neurodegeneration in methylmalonic aciduria involves inhibition of complex II and the tricarboxylic acid cycle, and synergistically acting excitotoxicity.
Farkas, LM; Feyh, P; Hinz, A; Hoffmann, GF; Hörster, F; Kölker, S; Mayatepek, E; Okun, JG; Sauer, S; Unsicker, K, 2002)		0.63
" Oxidative stress and excitotoxicity have been involved in the toxic pattern exerted by these organic acids."( The effect of WIN 55,212-2 suggests a cannabinoid-sensitive component in the early toxicity induced by organic acids accumulating in glutaric acidemia type I and in related disorders of propionate metabolism in rat brain synaptosomes.
Colín-González, AL; Leipnitz, G; Paz-Loyola, AL; Ribeiro, CA; Santamaría, A; Seminotti, B; Serratos, IN; Souza, DO; Wajner, M, 2015)		0.42

Pharmacokinetics

The purpose of this study was to investigate the utility of plasma pharmacokinetic and pharmacodynamic measures including plasma deoxynucleosides, homocysteine and methylmalonic acid concentrations in understanding the time course and extent of the inhibition of thymidylate synthase (TS) by pemetrexed.

ExcerptReferenceRelevance
"The purpose of this study was to investigate the utility of plasma pharmacokinetic and pharmacodynamic measures including plasma deoxynucleosides, homocysteine and methylmalonic acid concentrations in understanding the time course and extent of the inhibition of thymidylate synthase (TS) by pemetrexed in the context of a phase I/II combination study with vinorelbine."( Pemetrexed pharmacokinetics and pharmacodynamics in a phase I/II study of doublet chemotherapy with vinorelbine: implications for further optimisation of pemetrexed schedules.
Clarke, SJ; Li, KM; Rivory, LP, 2007)		0.54
" Weight and methylmalonic acid (MMA) level were predictive of pharmacokinetic (PK) variability."( A population pharmacokinetic and pharmacodynamic evaluation of pralatrexate in patients with relapsed or refractory non-Hodgkin's or Hodgkin's lymphoma.
Duffull, SB; Fleisher, M; Hamlin, P; Horwitz, S; Mould, DR; Neylon, E; O'Connor, OA; Saunders, ME; Sirotnak, F; Sweeney, K, 2009)		0.73

Compound-Compound Interactions

Methylmalonic aciduria combined with homocystinuria (MMA-HC) is a metabolic disorder resulting from impaired conversion of dietary cobalamin (cbl, or vitamin B12) to its two metabolically active forms.

ExcerptReferenceRelevance
"Number of patients with gastric body atrophy identified with the combination of MMA and Hcy, and pepsinogen A combined with pepsinogen C or gastrin."( Advantages of serum pepsinogen A combined with gastrin or pepsinogen C as first-line analytes in the evaluation of suspected cobalamin deficiency: a study in patients previously not subjected to gastrointestinal surgery.
Kilander, AF; Lindgren, A; Lindstedt, G, 1998)		0.3
" Serum pepsinogen A combined with pepsinogen C identified 100%, and combined with gastrin 88%, of the patients with gastric body atrophy and elevated metabolite tests, and 67 and 75%, respectively, of those who had not yet developed elevated metabolite tests."( Advantages of serum pepsinogen A combined with gastrin or pepsinogen C as first-line analytes in the evaluation of suspected cobalamin deficiency: a study in patients previously not subjected to gastrointestinal surgery.
Kilander, AF; Lindgren, A; Lindstedt, G, 1998)		0.3
"Pepsinogen A, combined with pepsinogen C or gastrin, should be the first option in evaluating patients with suspected cobalamin deficiency who have not previously undergone gastrointestinal surgery."( Advantages of serum pepsinogen A combined with gastrin or pepsinogen C as first-line analytes in the evaluation of suspected cobalamin deficiency: a study in patients previously not subjected to gastrointestinal surgery.
Kilander, AF; Lindgren, A; Lindstedt, G, 1998)		0.3
"Methylmalonic aciduria combined with homocystinuria (MMA-HC) is the biochemical trait of a metabolic disorder resulting from impaired conversion of dietary cobalamin (cbl, or vitamin B12) to its two metabolically active forms."( Increase in urinary purines and pyrimidines in patients with methylmalonic aciduria combined with homocystinuria.
Contini, L; Corda, M; Era, B; Fais, A; Lilliu, F; Porcu, S; Traldi, P, 2010)		2.04
"Urine samples were collected from three patients with methylmalonic aciduria combined with homocystinuria and from 70 healthy subjects."( Increase in urinary purines and pyrimidines in patients with methylmalonic aciduria combined with homocystinuria.
Contini, L; Corda, M; Era, B; Fais, A; Lilliu, F; Porcu, S; Traldi, P, 2010)		0.85
"An increased reactive oxygen species (ROS) production and apoptosis rate have been associated with several disorders involved in cobalamin metabolism, including isolated methylmalonic aciduria (MMA) cblB type and MMA combined with homocystinuria (MMAHC) cblC type."( Different altered pattern expression of genes related to apoptosis in isolated methylmalonic aciduria cblB type and combined with homocystinuria cblC type.
Gámez, A; Jorge-Finnigan, A; Pérez, B; Richard, E; Ugarte, M, 2010)		0.78

Bioavailability

ExcerptReferenceRelevance
"To clarify the bioavailability of vitamin B12 in lyophylized purple laver (nori; Porphyra yezoensis), total vitamin B12 and vitamin B12 analogue contents in the laver were determined, and the effects of feeding the laver to vitamin B12-deficient rats were investigated."( Feeding dried purple laver (nori) to vitamin B12-deficient rats significantly improves vitamin B12 status.
Abe, K; Ebara, S; Miyamoto, E; Nakano, Y; Sugiyama, S; Takenaka, S; Tamura, Y; Tsuyama, S; Watanabe, F, 2001)		0.31
" Little is known about the relative bioavailability of crystalline cobalamin added to food products."( Effect of supplementation with cobalamin carried either by a milk product or a capsule in mildly cobalamin-deficient elderly Dutch persons.
Blom, HJ; de Groot, LC; Dhonukshe-Rutten, RA; Eussen, SJ; van Staveren, WA; van Zutphen, M, 2005)		0.33
"Lambs with concentrations of vitamin B12 in plasma and liver in the marginal reference range were able to metabolise propionate and maintain normal concentrations of MMA in plasma at a rate of absorption of propionate close to that which would depress appetite."( Vitamin B12 absorption and metabolism in milk-fed lambs.
Furlong, JM; Gruner, TM; Sedcole, JR; Sykes, AR, 2009)		0.35
" Finally, amnionless is a novel biomarker which can detect perturbed vitamin B12 bioavailability during aging and physical frailty."( Vitamin B12 deficiency and impaired expression of amnionless during aging.
Bosco, N; De Castro, A; Feige, JN; Goulet, L; Karaz, S; Larbi, A; Michaud, J; Migliavacca, E; Ng, TP; Pannérec, A; Rezzi, S, 2018)		0.48

Dosage Studied

ExcerptRelevanceReference
" In addition a dose-response effect of alanine was observed."( Alanine decreases the protein requirements of infants with inborn errors of amino acid metabolism.
Algert, S; Kelts, DG; Nyhan, WL; Prodanos, C; Wolff, JA, 1985)		0.27
" There was a strong dose-response relationship between midtrimester serum MMA level and the risk for an NTD-affected pregnancy, with the relative risk increasing 13-fold for women with MMA levels > 90th percentile."( Elevated midtrimester serum methylmalonic acid levels as a risk factor for neural tube defects.
Adams, MJ; Cheek, JE; Haddow, JE; Henry, JP; Khoury, MJ; Knight, GJ; Scanlon, KS; Stabler, SP; Stevenson, RE; Sylvester, GC, 1995)		0.59
" Vitamin B12-depleted rats expired 16% of dosed 75Se-selenite as DMSe compared to 45% for control rats and excreted less TMSe in the urine (6."( Effect of vitamin B12 status on selenium methylation and toxicity in rats: in vivo and in vitro studies.
Chen, CL; Whanger, PD, 1993)		0.29
" The dosage of these two substances help to differenciate between both carencies, but it is not determinant of any of them and is an expensive method."( [Megaloblastic anemia: rapid and economical study].
Cicchetti, G; Marín, GH; Tentoni, J, 1997)		0.3
" Food folate intake had an inverse dose-response relation with tHcy that was limited to nonusers of supplements."( Association of folate intake and serum homocysteine in elderly persons according to vitamin supplementation and alcohol use.
Allen, RH; Baumgartner, RN; Garry, PJ; Koehler, KM; Rimm, EB; Stabler, SP, 2001)		0.31
" The relationship between Cbl supplement dosage and the biochemical parameters was dose dependent."( Is low-dose oral cobalamin enough to normalize cobalamin function in older people?
Day, A; Evans, L; Freedman, M; Garcia, A; Paris-Pombo, A, 2002)		0.31
"Serum MMA, tHcy, and other metabolites at baseline and after each 6-week dosing interval."( Response of elevated methylmalonic acid to three dose levels of oral cobalamin in older adults.
Allen, RH; Beresford, SA; Brodkin, KI; Rajan, S; Stabler, SP; Wallace, JI, 2002)		0.63
" Serum vitamin B12 was related to BMD in dose-response fashion up to about 200 pmol/L, and subjects with serum Hcy > or = 20 micromol/L had significantly lower BMD than subjects with serum Hcy < 10 micromol/L."( Relation between homocysteine and B-vitamin status indicators and bone mineral density in older Americans.
Jacques, PF; Morris, MS; Selhub, J, 2005)		0.33
" Cumulative lifetime L-dopa dosage and fasting MMA levels were associated with PN severity."( Neuropathy as a potential complication of levodopa use in Parkinson's disease.
Brown, MS; Furtado, S; Suchowersky, O; Toth, C; Zochodne, D, 2008)		0.35
", weight or body surface area)-based dosing and that pretreatment with folic acid and vitamin B(12) might diminish the incidence and severity of mucositis."( A population pharmacokinetic and pharmacodynamic evaluation of pralatrexate in patients with relapsed or refractory non-Hodgkin's or Hodgkin's lymphoma.
Duffull, SB; Fleisher, M; Hamlin, P; Horwitz, S; Mould, DR; Neylon, E; O'Connor, OA; Saunders, ME; Sirotnak, F; Sweeney, K, 2009)		0.35
" The dose-response curve also shows a lower effectivity of OHCbl with respect to stabilization, with an AC(50) of 7 microM."( Thermolability of mutant MMACHC protein in the vitamin B12-responsive cblC disorder.
Froese, DS; Gravel, RA; Healy, S; Kochan, G; McDonald, M; Niesen, FH; Oppermann, U, 2010)		0.36
" We discuss the importance of methylmalonic acid dosage after bariatric surgery in patients who present suspect of cyanocobalamin deficiency with normal values of this vitamin and the role of proton pump inhibitor use and vitamin supplementation in patients with early neurological presentation."( The importance of methylmalonic acid dosage on the assessment of patients with neurological manifestations following bariatric surgery.
Cosmo, C; Lucena, R; Vieira, C, 2011)		0.99
" A generalised additive regression model showed a positive dose-response relationship between the m-MMSE and choline (P=0·012 from a corresponding linear regression model)."( Plasma free choline, betaine and cognitive performance: the Hordaland Health Study.
Bjelland, I; Drevon, CA; Engedal, K; Nurk, E; Nygaard, HA; Refsum, H; Smith, DA; Tell, GS; Ueland, PM; Vollset, SE, 2013)		0.39
" The increase in the dosage of this gene results in an altered profile of metabolites involved in the folate pathway, including reduced homocysteine (Hcy), methionine, S-adenosylhomocysteine (SAH) and S-adenosylmethionine (SAM)."( Genetic polymorphisms modulate the folate metabolism of Brazilian individuals with Down syndrome.
Biselli, JM; Carvalho, VM; Eberlin, MN; Fonseca, MF; Goloni-Bertollo, EM; Haddad, R; Pavarino, EC; Vannucchi, H; Zampieri, BL, 2012)		0.38
" The authors summarized dose-response evidence from randomized controlled trials and prospective cohort studies on the relation of vitamin B12 intake and status with cognitive function in adults and elderly people."( Vitamin B12 intake and status and cognitive function in elderly people.
Brzozowska, A; Cavelaars, AE; de Groot, LC; Dhonukshe-Rutten, RA; Doets, EL; Dullemeijer, C; Souverein, OW; Szczecińska, A; van 't Veer, P; van Wijngaarden, JP, 2013)		0.39
" The use of these data to estimate dose-response relations provides a useful means to summarize the body of evidence."( Systematic review with dose-response meta-analyses between vitamin B-12 intake and European Micronutrient Recommendations Aligned's prioritized biomarkers of vitamin B-12 including randomized controlled trials and observational studies in adults and elder
Cavelaars, AE; de Boer, WJ; de Groot, LC; Dhonukshe-Rutten, RA; Doets, EL; Dullemeijer, C; In 't Veld, PH; Plada, M; Souverein, OW; van 't Veer, P; van der Voet, H; van Wijngaarden, JP, 2013)		0.39
"We systematically reviewed studies that investigated vitamin B-12 intake and biomarkers of vitamin B-12 status and estimated dose-response relations with the use of a meta-analysis."( Systematic review with dose-response meta-analyses between vitamin B-12 intake and European Micronutrient Recommendations Aligned's prioritized biomarkers of vitamin B-12 including randomized controlled trials and observational studies in adults and elder
Cavelaars, AE; de Boer, WJ; de Groot, LC; Dhonukshe-Rutten, RA; Doets, EL; Dullemeijer, C; In 't Veld, PH; Plada, M; Souverein, OW; van 't Veer, P; van der Voet, H; van Wijngaarden, JP, 2013)		0.39
"The meta-analysis of observational studies showed a weaker slope of dose-response relations than the meta-analysis of RCTs."( Systematic review with dose-response meta-analyses between vitamin B-12 intake and European Micronutrient Recommendations Aligned's prioritized biomarkers of vitamin B-12 including randomized controlled trials and observational studies in adults and elder
Cavelaars, AE; de Boer, WJ; de Groot, LC; Dhonukshe-Rutten, RA; Doets, EL; Dullemeijer, C; In 't Veld, PH; Plada, M; Souverein, OW; van 't Veer, P; van der Voet, H; van Wijngaarden, JP, 2013)		0.39
"The obtained dose-response estimate between vitamin B-12 intake and status provides complementary evidence to underpin recommendations for a vitamin B-12 intake of populations."( Systematic review with dose-response meta-analyses between vitamin B-12 intake and European Micronutrient Recommendations Aligned's prioritized biomarkers of vitamin B-12 including randomized controlled trials and observational studies in adults and elder
Cavelaars, AE; de Boer, WJ; de Groot, LC; Dhonukshe-Rutten, RA; Doets, EL; Dullemeijer, C; In 't Veld, PH; Plada, M; Souverein, OW; van 't Veer, P; van der Voet, H; van Wijngaarden, JP, 2013)		0.39
"2% completed their planned dosage within a 9-week feasibility time frame."( Reduced folate and serum vitamin metabolites in patients with rectal carcinoma: an open-label feasibility study of pemetrexed with folic acid and vitamin B12 supplementation.
Björkqvist, HG; Carlsson, GU; Gustavsson, BG; Kurlberg, GK; Odin, EA; Stoffregen, CC; Tångefjord, MT, 2016)		0.43
" She was maintained on 2 mg/kg/day dosing of hydroxocobalamin."( Clinical and biochemical outcomes in cobalamin C deficiency with use of high-dose hydroxocobalamin in the early neonatal period.
Farach, L; Frigeni, M; Gunther, K; Kacpura, A, 2022)		0.72
[information is derived through text-mining from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Roles (1)

RoleDescription
human metaboliteAny mammalian metabolite produced during a metabolic reaction in humans (Homo sapiens).
[role information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Drug Classes (1)

ClassDescription
C4-dicarboxylic acidAny dicarboxylic acid that contains four carbon atoms.
[compound class information is derived from Chemical Entities of Biological Interest (ChEBI), Hastings J, Owen G, Dekker A, Ennis M, Kale N, Muthukrishnan V, Turner S, Swainston N, Mendes P, Steinbeck C. (2016). ChEBI in 2016: Improved services and an expanding collection of metabolites. Nucleic Acids Res]

Pathways (28)

PathwayProteinsCompounds
Valine, Leucine, and Isoleucine Degradation2852
Propanoate Metabolism1837
beta-Ketothiolase Deficiency2852
2-Methyl-3-hydroxybutyryl-CoA Dehydrogenase Deficiency2852
Propionic Acidemia2852
3-Hydroxy-3-methylglutaryl-CoA Lyase Deficiency2852
Maple Syrup Urine Disease2852
3-Methylcrotonyl-CoA Carboxylase Deficiency Type I2852
3-Methylglutaconic Aciduria Type I2852
3-Methylglutaconic Aciduria Type III2852
Methylmalonate Semialdehyde Dehydrogenase Deficiency2852
Methylmalonic Aciduria2852
Isovaleric Aciduria2852
Malonic Aciduria1837
Methylmalonic Aciduria Due to Cobalamin-Related Disorders1837
3-Methylglutaconic Aciduria Type IV2852
Malonyl-CoA Decarboxylase Deficiency1837
3-Hydroxyisobutyric Acid Dehydrogenase Deficiency2852
3-Hydroxyisobutyric Aciduria2852
Isobutyryl-CoA Dehydrogenase Deficiency2852
Isovaleric Acidemia2852
2-Methyl-3-hydroxybutryl-CoA Dehydrogenase Deficiency2952
Valine,Leucine and Isoleucine degradation ( Valine,Leucine and Isoleucine degradation )2936
Leucine, isoleucine and valine metabolism2470
Folic acid network070
Valine, leucine and isoleucine degradation034
Vitamin B12 metabolism050
Krebs cycle disorders1214

Protein Targets (11)

Potency Measurements

ProteinTaxonomyMeasurementAverage (µ)Min (ref.)Avg (ref.)Max (ref.)Bioassay(s)
Chain A, Beta-lactamaseEscherichia coli K-12Potency5.62340.044717.8581100.0000AID485294
Chain A, JmjC domain-containing histone demethylation protein 3AHomo sapiens (human)Potency79.43280.631035.7641100.0000AID504339
euchromatic histone-lysine N-methyltransferase 2Homo sapiens (human)Potency10.00000.035520.977089.1251AID504332
importin subunit beta-1 isoform 1Homo sapiens (human)Potency0.29095.804836.130665.1308AID540253
snurportin-1Homo sapiens (human)Potency0.29095.804836.130665.1308AID540253
peptidyl-prolyl cis-trans isomerase NIMA-interacting 1Homo sapiens (human)Potency39.81070.425612.059128.1838AID504891
tumor susceptibility gene 101 proteinHomo sapiens (human)Potency56.23410.129810.833132.6090AID493005
GTP-binding nuclear protein Ran isoform 1Homo sapiens (human)Potency0.29095.804816.996225.9290AID540253
gemininHomo sapiens (human)Potency0.63100.004611.374133.4983AID624297
Guanine nucleotide-binding protein GHomo sapiens (human)Potency25.11891.995325.532750.1187AID624288
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Inhibition Measurements

ProteinTaxonomyMeasurementAverageMin (ref.)Avg (ref.)Max (ref.)Bioassay(s)
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Biological Processes (5)

Processvia Protein(s)Taxonomy
negative regulation of inflammatory response to antigenic stimulusGuanine nucleotide-binding protein GHomo sapiens (human)
renal water homeostasisGuanine nucleotide-binding protein GHomo sapiens (human)
G protein-coupled receptor signaling pathwayGuanine nucleotide-binding protein GHomo sapiens (human)
regulation of insulin secretionGuanine nucleotide-binding protein GHomo sapiens (human)
cellular response to glucagon stimulusGuanine nucleotide-binding protein GHomo sapiens (human)
[Information is prepared from geneontology information from the June-17-2024 release]

Molecular Functions (2)

Processvia Protein(s)Taxonomy
G protein activityGuanine nucleotide-binding protein GHomo sapiens (human)
adenylate cyclase activator activityGuanine nucleotide-binding protein GHomo sapiens (human)
[Information is prepared from geneontology information from the June-17-2024 release]

Ceullar Components (1)

Processvia Protein(s)Taxonomy
plasma membraneGuanine nucleotide-binding protein GHomo sapiens (human)
[Information is prepared from geneontology information from the June-17-2024 release]

Bioassays (16)

Assay IDTitleYearJournalArticle
AID1745845Primary qHTS for Inhibitors of ATXN expression
AID588499High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain A protease, MLPCN compound set2010Current protocols in cytometry, Oct, Volume: Chapter 13Microsphere-based flow cytometry protease assays for use in protease activity detection and high-throughput screening.
AID588499High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain A protease, MLPCN compound set2006Cytometry. Part A : the journal of the International Society for Analytical Cytology, May, Volume: 69, Issue:5
Microsphere-based protease assays and screening application for lethal factor and factor Xa.
AID588499High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain A protease, MLPCN compound set2010Assay and drug development technologies, Feb, Volume: 8, Issue:1
High-throughput multiplex flow cytometry screening for botulinum neurotoxin type a light chain protease inhibitors.
AID588501High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Lethal Factor Protease, MLPCN compound set2010Current protocols in cytometry, Oct, Volume: Chapter 13Microsphere-based flow cytometry protease assays for use in protease activity detection and high-throughput screening.
AID588501High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Lethal Factor Protease, MLPCN compound set2006Cytometry. Part A : the journal of the International Society for Analytical Cytology, May, Volume: 69, Issue:5
Microsphere-based protease assays and screening application for lethal factor and factor Xa.
AID588501High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Lethal Factor Protease, MLPCN compound set2010Assay and drug development technologies, Feb, Volume: 8, Issue:1
High-throughput multiplex flow cytometry screening for botulinum neurotoxin type a light chain protease inhibitors.
AID588497High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain F protease, MLPCN compound set2010Current protocols in cytometry, Oct, Volume: Chapter 13Microsphere-based flow cytometry protease assays for use in protease activity detection and high-throughput screening.
AID588497High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain F protease, MLPCN compound set2006Cytometry. Part A : the journal of the International Society for Analytical Cytology, May, Volume: 69, Issue:5
Microsphere-based protease assays and screening application for lethal factor and factor Xa.
AID588497High-throughput multiplex microsphere screening for inhibitors of toxin protease, specifically Botulinum neurotoxin light chain F protease, MLPCN compound set2010Assay and drug development technologies, Feb, Volume: 8, Issue:1
High-throughput multiplex flow cytometry screening for botulinum neurotoxin type a light chain protease inhibitors.
AID651635Viability Counterscreen for Primary qHTS for Inhibitors of ATXN expression
AID504812Inverse Agonists of the Thyroid Stimulating Hormone Receptor: HTS campaign2010Endocrinology, Jul, Volume: 151, Issue:7
A small molecule inverse agonist for the human thyroid-stimulating hormone receptor.
AID504810Antagonists of the Thyroid Stimulating Hormone Receptor: HTS campaign2010Endocrinology, Jul, Volume: 151, Issue:7
A small molecule inverse agonist for the human thyroid-stimulating hormone receptor.
AID1174256Inhibition of mouse serine racemase using L-serine substrate by reversed-phase HPLC analysis2015European journal of medicinal chemistry, Jan-07, Volume: 89Malonate-based inhibitors of mammalian serine racemase: kinetic characterization and structure-based computational study.
AID1345450Human succinate receptor (Succinate receptor)2017British journal of pharmacology, 05, Volume: 174, Issue:9
Identification and pharmacological characterization of succinate receptor agonists.
AID1345450Human succinate receptor (Succinate receptor)2004Nature, May-13, Volume: 429, Issue:6988
Citric acid cycle intermediates as ligands for orphan G-protein-coupled receptors.
[information is prepared from bioassay data collected from National Library of Medicine (NLM), extracted Dec-2023]

Research

Studies (1,652)

TimeframeStudies, This Drug (%)All Drugs %
pre-1990324 (19.61)18.7374
1990's343 (20.76)18.2507
2000's472 (28.57)29.6817
2010's381 (23.06)24.3611
2020's132 (7.99)2.80
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Market Indicators

Research Demand Index: 89.81

According to the monthly volume, diversity, and competition of internet searches for this compound, as well the volume and growth of publications, there is estimated to be very strong demand-to-supply ratio for research on this compound.

MetricThis Compound (vs All)
Research Demand Index89.81 (24.57)
Research Supply Index7.50 (2.92)
Research Growth Index4.60 (4.65)
Search Engine Demand Index164.74 (26.88)
Search Engine Supply Index2.01 (0.95)

This Compound (89.81)

All Compounds (24.57)

Study Types

Publication TypeThis drug (%)All Drugs (%)
Trials90 (5.23%)5.53%
Reviews159 (9.23%)6.00%
Case Studies371 (21.54%)4.05%
Observational7 (0.41%)0.25%
Other1,095 (63.59%)84.16%
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]
SubstanceRelationship StrengthStudiesTrialsClassesRoles
acetylcarnitine
Acetylcarnitine: An acetic acid ester of CARNITINE that facilitates movement of ACETYL COA into the matrices of mammalian MITOCHONDRIA during the oxidation of FATTY ACIDS.		5120O-acylcarnitinehuman metabolite
ethylene chlorohydrin
Ethylene Chlorohydrin: Used as a solvent, in the manufacture of insecticides, and for treating sweet potatoes before planting. May cause nausea, vomiting, pains in head and chest, stupefaction. Irritates mucous membranes and causes kidney and liver degeneration.. chloroethanol : An organochlorine compound that is ethanol substituted by at least one chloro group.		2.4520chloroethanolxenobiotic metabolite
alpha-hydroxyglutarate
2-hydroxyglutarate : A dicarboxylic acid anion obtained by deprotonation of at least one of the carboxy groups of 2-hydroxyglutaric acid.. 2-hydroxyglutaric acid : A 2-hydroxydicarboxylic acid that is glutaric acid in which one hydrogen alpha- to a carboxylic acid group is substituted by a hydroxy group.		3.51202-hydroxydicarboxylic acid;
dicarboxylic fatty acid		metabolite;
mouse metabolite
tartronic acid
hydroxymalonic acid : A dicarboxylic acid that is malonic acid substituted by a hydroxy group at position 2.		2.1110dicarboxylic acid;
dicarboxylic fatty acid		plant metabolite
alpha-ketoglutaric acid
2-oxoglutaric acid : An oxo dicarboxylic acid that consists of glutaric acid bearing an oxo substituent at position 2. It is an intermediate metabolite in Krebs cycle.		2.9310oxo dicarboxylic acidfundamental metabolite
alpha-ketobutyric acid
alpha-ketobutyric acid: RN given refers to parent cpd; structure. 2-oxobutanoic acid : A 2-oxo monocarboxylic acid that is the 2-oxo derivative of butanoic acid.		2102-oxo monocarboxylic acid;
short-chain fatty acid
2-keto-4-methylvalerate
alpha-ketoisocaproic acid: RN given refers to parent cpd. 4-methyl-2-oxopentanoate : A 2-oxo monocarboxylic acid anion that is the conjugate base of 4-methyl-2-oxopentanoic acid.. 4-methyl-2-oxopentanoic acid : A 2-oxo monocarboxylic acid that is pentanoic acid (valeric acid) substituted with a keto group at C-2 and a methyl group at C-4. A metabolite that has been found to accumulate in maple syrup urine disease.		2102-oxo monocarboxylic acid;
branched-chain keto acid		algal metabolite;
human metabolite
3-hydroxyisobutyric acid
3-hydroxyisobutyric acid: RN given refers to cpd without isomeric designation. 3-hydroxyisobutyric acid : A 4-carbon, branched hydroxy fatty acid and intermediate in the metabolism of valine.		2.46203-hydroxy monocarboxylic acid
acetoacetic acid
acetoacetic acid : A 3-oxo monocarboxylic acid that is butyric acid bearing a 3-oxo substituent.		1.97103-oxo fatty acid;
ketone body		metabolite
gamma-aminobutyric acid
gamma-Aminobutyric Acid: The most common inhibitory neurotransmitter in the central nervous system.. gamma-aminobutyric acid : A gamma-amino acid that is butanoic acid with the amino substituent located at C-4.		3.7730amino acid zwitterion;
gamma-amino acid;
monocarboxylic acid		human metabolite;
neurotransmitter;
Saccharomyces cerevisiae metabolite;
signalling molecule
4-hydroxyphenylacetic acid
4-hydroxyphenylacetic acid : A monocarboxylic acid that is acetic acid in which one of the methyl hydrogens is substituted by a 4-hydroxyphenyl group.		2.3820monocarboxylic acid;
phenols		fungal metabolite;
human metabolite;
mouse metabolite;
plant metabolite
4-hydroxybenzoic acid
4-hydroxybenzoic acid : A monohydroxybenzoic acid that is benzoic acid carrying a hydroxy substituent at C-4 of the benzene ring.		210monohydroxybenzoic acidalgal metabolite;
plant metabolite
ethylene glycol
Ethylene Glycol: A colorless, odorless, viscous dihydroxy alcohol. It has a sweet taste, but is poisonous if ingested. Ethylene glycol is the most important glycol commercially available and is manufactured on a large scale in the United States. It is used as an antifreeze and coolant, in hydraulic fluids, and in the manufacture of low-freezing dynamites and resins.. ethanediol : Any diol that is ethane or substituted ethane carrying two hydroxy groups.. ethylene glycol : A 1,2-glycol compound produced via reaction of ethylene oxide with water.		7.3820ethanediol;
glycol		metabolite;
mouse metabolite;
solvent;
toxin
acetic acid
Acetic Acid: Product of the oxidation of ethanol and of the destructive distillation of wood. It is used locally, occasionally internally, as a counterirritant and also as a reagent. (Stedman, 26th ed). acetic acid : A simple monocarboxylic acid containing two carbons.		2.4320monocarboxylic acidantimicrobial food preservative;
Daphnia magna metabolite;
food acidity regulator;
protic solvent
acetoin
[no description available]		1.9710methyl ketone;
secondary alpha-hydroxy ketone		metabolite
acetone
methyl ketone : A ketone of formula RC(=O)CH3 (R =/= H).		2.0610ketone body;
methyl ketone;
propanones;
volatile organic compound		EC 3.5.1.4 (amidase) inhibitor;
human metabolite;
polar aprotic solvent
adipic acid
adipic acid : An alpha,omega-dicarboxylic acid that is the 1,4-dicarboxy derivative of butane.		2.3920alpha,omega-dicarboxylic acid;
dicarboxylic fatty acid		food acidity regulator;
human xenobiotic metabolite
ammonium hydroxide
azane : Saturated acyclic nitrogen hydrides having the general formula NnHn+2.		6.19260azane;
gas molecular entity;
mononuclear parent hydride		EC 3.5.1.4 (amidase) inhibitor;
metabolite;
mouse metabolite;
neurotoxin;
NMR chemical shift reference compound;
nucleophilic reagent;
refrigerant
benzene
[no description available]		2.610aromatic annulene;
benzenes;
volatile organic compound		carcinogenic agent;
environmental contaminant;
non-polar solvent
betaine
glycine betaine : The amino acid betaine derived from glycine.		11.16170amino-acid betaine;
glycine derivative		fundamental metabolite
2,3-butylene glycol
2,3-butylene glycol: RN given refers to cpd without isomeric designation. butane-2,3-diol : A butanediol in which hydroxylation is at C-2 and C-3.		1.9710butanediol;
glycol;
secondary alcohol
butyric acid
Butyric Acid: A four carbon acid, CH3CH2CH2COOH, with an unpleasant odor that occurs in butter and animal fat as the glycerol ester.. butyrate : A short-chain fatty acid anion that is the conjugate base of butyric acid, obtained by deprotonation of the carboxy group.. butyric acid : A straight-chain saturated fatty acid that is butane in which one of the terminal methyl groups has been oxidised to a carboxy group.		210fatty acid 4:0;
straight-chain saturated fatty acid		human urinary metabolite;
Mycoplasma genitalium metabolite
cadaverine
[no description available]		1.9810alkane-alpha,omega-diamineDaphnia magna metabolite;
Escherichia coli metabolite;
mouse metabolite;
plant metabolite
formic acid
formic acid: RN given refers to parent cpd. formic acid : The simplest carboxylic acid, containing a single carbon. Occurs naturally in various sources including the venom of bee and ant stings, and is a useful organic synthetic reagent. Principally used as a preservative and antibacterial agent in livestock feed. Induces severe metabolic acidosis and ocular injury in human subjects.		3.360monocarboxylic acidantibacterial agent;
astringent;
metabolite;
protic solvent;
solvent
carnitine
[no description available]		11.59804amino-acid betainehuman metabolite;
mouse metabolite
methane
Methane: The simplest saturated hydrocarbon. It is a colorless, flammable gas, slightly soluble in water. It is one of the chief constituents of natural gas and is formed in the decomposition of organic matter. (Grant & Hackh's Chemical Dictionary, 5th ed). methane : A one-carbon compound in which the carbon is attached by single bonds to four hydrogen atoms. It is a colourless, odourless, non-toxic but flammable gas (b.p. -161degreeC).		2.2510alkane;
gas molecular entity;
mononuclear parent hydride;
one-carbon compound		bacterial metabolite;
fossil fuel;
greenhouse gas
choline
[no description available]		6.7173cholinesallergen;
Daphnia magna metabolite;
Escherichia coli metabolite;
human metabolite;
mouse metabolite;
neurotransmitter;
nutrient;
plant metabolite;
Saccharomyces cerevisiae metabolite
citric acid, anhydrous
Citric Acid: A key intermediate in metabolism. It is an acid compound found in citrus fruits. The salts of citric acid (citrates) can be used as anticoagulants due to their calcium chelating ability.. citric acid : A tricarboxylic acid that is propane-1,2,3-tricarboxylic acid bearing a hydroxy substituent at position 2. It is an important metabolite in the pathway of all aerobic organisms.		2.9340tricarboxylic acidantimicrobial agent;
chelator;
food acidity regulator;
fundamental metabolite
chlorine
chloride : A halide anion formed when chlorine picks up an electron to form an an anion.		2.3920halide anion;
monoatomic chlorine		cofactor;
Escherichia coli metabolite;
human metabolite
coumarin
2H-chromen-2-one: coumarin derivative		2.110coumarinsfluorescent dye;
human metabolite;
plant metabolite
3-hydroxybutyric acid
3-Hydroxybutyric Acid: BUTYRIC ACID substituted in the beta or 3 position. It is one of the ketone bodies produced in the liver.. 3-hydroxybutyric acid : A straight-chain 3-hydroxy monocarboxylic acid comprising a butyric acid core with a single hydroxy substituent in the 3- position; a ketone body whose levels are raised during ketosis, used as an energy source by the brain during fasting in humans. Also used to synthesise biodegradable plastics.		3.1150(omega-1)-hydroxy fatty acid;
3-hydroxy monocarboxylic acid;
hydroxybutyric acid		human metabolite
n(1)-methylnicotinamide
N(1)-methylnicotinamide: RN given refers to parent cpd. 1-methylnicotinamide : A pyridinium ion comprising nicotinamide having a methyl group at the 1-position. It is a metabolite of nicotinamide which was initially considered to be biologically inactive but has emerged as an anti-thrombotic and anti-inflammatory agent.		2.0610pyridinium ionalgal metabolite;
anti-inflammatory agent;
human urinary metabolite;
mouse metabolite;
plant metabolite;
Saccharomyces cerevisiae metabolite
2-methylcitric acid
2-methylcitric acid: RN given refers to parent cpd		7.92231tricarboxylic acid
malic acid
malic acid : A 2-hydroxydicarboxylic acid that is succinic acid in which one of the hydrogens attached to a carbon is replaced by a hydroxy group.. 2-hydroxydicarboxylic acid : Any dicarboxylic acid carrying a hydroxy group on the carbon atom at position alpha to the carboxy group.		2.4202-hydroxydicarboxylic acid;
C4-dicarboxylic acid		food acidity regulator;
fundamental metabolite
propionaldehyde
propionaldehyde: may cause respiratory irritation; RN given refers to parent cpd; structure. propanal : An aldehyde that consists of ethane bearing a formyl substituent. The parent of the class of propanals.		2.0610alpha-CH2-containing aldehyde;
propanals		Escherichia coli metabolite
creatine
[no description available]		3.2560glycine derivative;
guanidines;
zwitterion		geroprotector;
human metabolite;
mouse metabolite;
neuroprotective agent;
nutraceutical
cytosine
[no description available]		2.0610aminopyrimidine;
pyrimidine nucleobase;
pyrimidone		Escherichia coli metabolite;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
lactic acid
Lactic Acid: A normal intermediate in the fermentation (oxidation, metabolism) of sugar. The concentrated form is used internally to prevent gastrointestinal fermentation. (From Stedman, 26th ed). 2-hydroxypropanoic acid : A 2-hydroxy monocarboxylic acid that is propanoic acid in which one of the alpha-hydrogens is replaced by a hydroxy group.		5.952002-hydroxy monocarboxylic acidalgal metabolite;
Daphnia magna metabolite
dimethylglycine
dimethylglycine: metabolic product of calcium pangamate; mutagen when mixed with nitrite; RN given refers to parent cpd. N,N-dimethylglycine : An N-methylglycine that is glycine carrying two N-methyl substituents.		4.732amino acid zwitterion;
N-methyl-amino acid;
N-methylglycines		Daphnia magna metabolite;
human metabolite;
mouse metabolite
dimethyl sulfoxide
Dimethyl Sulfoxide: A highly polar organic liquid, that is used widely as a chemical solvent. Because of its ability to penetrate biological membranes, it is used as a vehicle for topical application of pharmaceuticals. It is also used to protect tissue during CRYOPRESERVATION. Dimethyl sulfoxide shows a range of pharmacological activity including analgesia and anti-inflammation.. dimethyl sulfoxide : A 2-carbon sulfoxide in which the sulfur atom has two methyl substituents.		210sulfoxide;
volatile organic compound		alkylating agent;
antidote;
Escherichia coli metabolite;
geroprotector;
MRI contrast agent;
non-narcotic analgesic;
polar aprotic solvent;
radical scavenger
gamma-butyrobetaine
4-(trimethylammonio)butanoate : An amino-acid betaine gamma-aminobutyric acid zwitterion in which all of the hydrogens attached to the nitrogen are replaced by methyl groups.		2.0310amino-acid betaineEscherichia coli metabolite;
human metabolite
glutaric acid
glutaric acid: RN given refers to parent cpd. glutaric acid : An alpha,omega-dicarboxylic acid that is a linear five-carbon dicarboxylic acid.		5.83110alpha,omega-dicarboxylic acid;
dicarboxylic fatty acid		Daphnia magna metabolite;
human metabolite
glycine
[no description available]		7.68291alpha-amino acid;
amino acid zwitterion;
proteinogenic amino acid;
serine family amino acid		EC 2.1.2.1 (glycine hydroxymethyltransferase) inhibitor;
fundamental metabolite;
hepatoprotective agent;
micronutrient;
neurotransmitter;
NMDA receptor agonist;
nutraceutical
glycerol
Moon: The natural satellite of the planet Earth. It includes the lunar cycles or phases, the lunar month, lunar landscapes, geography, and soil.		1.9610alditol;
triol		algal metabolite;
detergent;
Escherichia coli metabolite;
geroprotector;
human metabolite;
mouse metabolite;
osmolyte;
Saccharomyces cerevisiae metabolite;
solvent
glycolic acid
glycolic acid: RN given refers to parent cpd. glycolic acid : A 2-hydroxy monocarboxylic acid that is acetic acid where the methyl group has been hydroxylated.		1.98102-hydroxy monocarboxylic acid;
primary alcohol		keratolytic drug;
metabolite
glycocyamine
glycocyamine: RN given refers to parent cpd; structure. guanidinoacetate : A monocarboxylic acid anion that is the conjugate base of guanidinoacetic acid, obtained by deprotonation of the carboxy group.. guanidinoacetic acid : The N-amidino derivative of glycine.		2.0210guanidinoacetic acids;
zwitterion		bacterial metabolite;
human metabolite;
mouse metabolite;
nutraceutical;
rat metabolite
hydrogen carbonate
Bicarbonates: Inorganic salts that contain the -HCO3 radical. They are an important factor in determining the pH of the blood and the concentration of bicarbonate ions is regulated by the kidney. Levels in the blood are an index of the alkali reserve or buffering capacity.. hydrogencarbonate : The carbon oxoanion resulting from the removal of a proton from carbonic acid.		3.2160carbon oxoanioncofactor;
Escherichia coli metabolite;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
hydrogen
Hydrogen: The first chemical element in the periodic table with atomic symbol H, and atomic number 1. Protium (atomic weight 1) is by far the most common hydrogen isotope. Hydrogen also exists as the stable isotope DEUTERIUM (atomic weight 2) and the radioactive isotope TRITIUM (atomic weight 3). Hydrogen forms into a diatomic molecule at room temperature and appears as a highly flammable colorless and odorless gas.. dihydrogen : An elemental molecule consisting of two hydrogens joined by a single bond.		3.3611elemental hydrogen;
elemental molecule;
gas molecular entity		antioxidant;
electron donor;
food packaging gas;
fuel;
human metabolite
thioctic acid
Thioctic Acid: An octanoic acid bridged with two sulfurs so that it is sometimes also called a pentanoic acid in some naming schemes. It is biosynthesized by cleavage of LINOLEIC ACID and is a coenzyme of oxoglutarate dehydrogenase (KETOGLUTARATE DEHYDROGENASE COMPLEX). It is used in DIETARY SUPPLEMENTS.		1.9510dithiolanes;
heterocyclic fatty acid;
thia fatty acid		fundamental metabolite;
geroprotector
malonic acid
malonic acid : An alpha,omega-dicarboxylic acid in which the two carboxy groups are separated by a single methylene group.. dicarboxylic acid : Any carboxylic acid containing two carboxy groups.		5.56220alpha,omega-dicarboxylic acidhuman metabolite
methanol
Methanol: A colorless, flammable liquid used in the manufacture of FORMALDEHYDE and ACETIC ACID, in chemical synthesis, antifreeze, and as a solvent. Ingestion of methanol is toxic and may cause blindness.. primary alcohol : A primary alcohol is a compound in which a hydroxy group, -OH, is attached to a saturated carbon atom which has either three hydrogen atoms attached to it or only one other carbon atom and two hydrogen atoms attached to it.. methanol : The primary alcohol that is the simplest aliphatic alcohol, comprising a methyl and an alcohol group.		6.9710alkyl alcohol;
one-carbon compound;
primary alcohol;
volatile organic compound		amphiprotic solvent;
Escherichia coli metabolite;
fuel;
human metabolite;
mouse metabolite;
Mycoplasma genitalium metabolite
melatonin
[no description available]		7.4110acetamides;
tryptamines		anticonvulsant;
central nervous system depressant;
geroprotector;
hormone;
human metabolite;
immunological adjuvant;
mouse metabolite;
radical scavenger
niacinamide
nicotinamide : A pyridinecarboxamide that is pyridine in which the hydrogen at position 3 is replaced by a carboxamide group.		2.4920pyridine alkaloid;
pyridinecarboxamide;
vitamin B3		anti-inflammatory agent;
antioxidant;
cofactor;
EC 2.4.2.30 (NAD(+) ADP-ribosyltransferase) inhibitor;
EC 3.5.1.98 (histone deacetylase) inhibitor;
Escherichia coli metabolite;
geroprotector;
human urinary metabolite;
metabolite;
mouse metabolite;
neuroprotective agent;
Saccharomyces cerevisiae metabolite;
Sir2 inhibitor
nitrates
Nitrates: Inorganic or organic salts and esters of nitric acid. These compounds contain the NO3- radical.		2.7730monovalent inorganic anion;
nitrogen oxoanion;
reactive nitrogen species
nitrites
Nitrites: Salts of nitrous acid or compounds containing the group NO2-. The inorganic nitrites of the type MNO2 (where M=metal) are all insoluble, except the alkali nitrites. The organic nitrites may be isomeric, but not identical with the corresponding nitro compounds. (Grant & Hackh's Chemical Dictionary, 5th ed)		2.4920monovalent inorganic anion;
nitrogen oxoanion;
reactive nitrogen species		human metabolite
nitrous oxide
Nitrous Oxide: Nitrogen oxide (N2O). A colorless, odorless gas that is used as an anesthetic and analgesic. High concentrations cause a narcotic effect and may replace oxygen, causing death by asphyxia. It is also used as a food aerosol in the preparation of whipping cream.. dinitrogen oxide : A nitrogen oxide consisting of linear unsymmetrical molecules with formula N2O. While it is the most used gaseous anaesthetic in the world, its major commercial use, due to its solubility under pressure in vegetable fats combined with its non-toxicity in low concentrations, is as an aerosol spray propellant and aerating agent for canisters of 'whipped' cream.		6.05150gas molecular entity;
nitrogen oxide		analgesic;
bacterial metabolite;
food packaging gas;
food propellant;
general anaesthetic;
greenhouse gas;
inhalation anaesthetic;
NMDA receptor antagonist;
raising agent;
refrigerant;
vasodilator agent
orotic acid
Orotic Acid: An intermediate product in PYRIMIDINE synthesis which plays a role in chemical conversions between DIHYDROFOLATE and TETRAHYDROFOLATE.. orotic acid : A pyrimidinemonocarboxylic acid that is uracil bearing a carboxy substituent at position C-6.		3.4720pyrimidinemonocarboxylic acidEscherichia coli metabolite;
metabolite;
mouse metabolite
oxaloacetic acid
Oxaloacetic Acid: A dicarboxylic acid ketone that is an important metabolic intermediate of the CITRIC ACID CYCLE. It can be converted to ASPARTIC ACID by ASPARTATE TRANSAMINASE.. oxaloacetic acid : An oxodicarboxylic acid that is succinic acid bearing a single oxo group.		210C4-dicarboxylic acid;
oxo dicarboxylic acid		geroprotector;
metabolite
oxalic acid
Oxalic Acid: A strong dicarboxylic acid occurring in many plants and vegetables. It is produced in the body by metabolism of glyoxylic acid or ascorbic acid. It is not metabolized but excreted in the urine. It is used as an analytical reagent and general reducing agent.. oxalic acid : An alpha,omega-dicarboxylic acid that is ethane substituted by carboxyl groups at positions 1 and 2.		2.6730alpha,omega-dicarboxylic acidalgal metabolite;
human metabolite;
plant metabolite
propionic acid
propionic acid : A short-chain saturated fatty acid comprising ethane attached to the carbon of a carboxy group.		7.16690saturated fatty acid;
short-chain fatty acid		antifungal drug
putrescine
[no description available]		210alkane-alpha,omega-diamineantioxidant;
fundamental metabolite
pyrazole
1H-pyrazole : The 1H-tautomer of pyrazole.		210pyrazole
pyridoxal
[no description available]		2.1310hydroxymethylpyridine;
methylpyridines;
monohydroxypyridine;
pyridinecarbaldehyde;
vitamin B6		cofactor;
Escherichia coli metabolite;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
pyridoxal phosphate
Pyridoxal Phosphate: This is the active form of VITAMIN B 6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (PYRIDOXAMINE).. pyridoxal 5'-phosphate : The monophosphate ester obtained by condensation of phosphoric acid with the primary hydroxy group of pyridoxal.		6.1232methylpyridines;
monohydroxypyridine;
pyridinecarbaldehyde;
vitamin B6 phosphate		coenzyme;
cofactor;
EC 2.7.7.7 (DNA-directed DNA polymerase) inhibitor;
Escherichia coli metabolite;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
pyridoxine
4,5-bis(hydroxymethyl)-2-methylpyridin-3-ol: structure in first source. vitamin B6 : Any member of the group of pyridines that exhibit biological activity against vitamin B6 deficiency. Vitamin B6 deficiency is associated with microcytic anemia, electroencephalographic abnormalities, dermatitis with cheilosis (scaling on the lips and cracks at the corners of the mouth) and glossitis (swollen tongue), depression and confusion, and weakened immune function. Vitamin B6 consists of the vitamers pyridoxine, pyridoxal, and pyridoxamine and their respective 5'-phosphate esters (and includes their corresponding ionized and salt forms).		7.0581hydroxymethylpyridine;
methylpyridines;
monohydroxypyridine;
vitamin B6		cofactor;
Escherichia coli metabolite;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
pyruvic acid
Pyruvic Acid: An intermediate compound in the metabolism of carbohydrates, proteins, and fats. In thiamine deficiency, its oxidation is retarded and it accumulates in the tissues, especially in nervous structures. (From Stedman, 26th ed). pyruvic acid : A 2-oxo monocarboxylic acid that is the 2-keto derivative of propionic acid. It is a metabolite obtained during glycolysis.		3.24602-oxo monocarboxylic acidcofactor;
fundamental metabolite
quinolinic acid
Quinolinic Acid: A metabolite of tryptophan with a possible role in neurodegenerative disorders. Elevated CSF levels of quinolinic acid are correlated with the severity of neuropsychological deficits in patients who have AIDS.. pyridinedicarboxylic acid : Any member of the class of pyridines carrying two carboxy groups.. quinolinic acid : A pyridinedicarboxylic acid that is pyridine substituted by carboxy groups at positions 2 and 3. It is a metabolite of tryptophan.		2.1110pyridinedicarboxylic acidEscherichia coli metabolite;
human metabolite;
mouse metabolite;
NMDA receptor agonist
sarcosine
cocobetaine: N-alkyl-betaine; cause of shampoo dermatitis		4.732N-alkylglycine zwitterion;
N-alkylglycine;
N-methyl-amino acid;
N-methylglycines		Escherichia coli metabolite;
glycine receptor agonist;
glycine transporter 1 inhibitor;
human metabolite;
mouse metabolite
sulfites
Sulfites: Inorganic salts of sulfurous acid.. sulfites : Any sulfurous acid derivative that is a salt or an ester of sulfurous acid.. organosulfonate oxoanion : An organic anion obtained by deprotonation of the sufonate group(s) of any organosulfonic acid.. sulfite : A sulfur oxoanion that is the conjugate base of hydrogen sulfite (H2SO3).		1.9610divalent inorganic anion;
sulfur oxide;
sulfur oxoanion
succinic acid
Succinic Acid: A water-soluble, colorless crystal with an acid taste that is used as a chemical intermediate, in medicine, the manufacture of lacquers, and to make perfume esters. It is also used in foods as a sequestrant, buffer, and a neutralizing agent. (Hawley's Condensed Chemical Dictionary, 12th ed, p1099; McGraw-Hill Dictionary of Scientific and Technical Terms, 4th ed, p1851). succinic acid : An alpha,omega-dicarboxylic acid resulting from the formal oxidation of each of the terminal methyl groups of butane to the corresponding carboxy group. It is an intermediate metabolite in the citric acid cycle.		6.5230alpha,omega-dicarboxylic acid;
C4-dicarboxylic acid		anti-ulcer drug;
fundamental metabolite;
micronutrient;
nutraceutical;
radiation protective agent
taurine
[no description available]		3.4720amino sulfonic acid;
zwitterion		antioxidant;
Escherichia coli metabolite;
glycine receptor agonist;
human metabolite;
mouse metabolite;
nutrient;
radical scavenger;
Saccharomyces cerevisiae metabolite
thiamine
thiamine(1+) : A primary alcohol that is 1,3-thiazol-3-ium substituted by (4-amino-2-methylpyrimidin-5-yl)methyl, methyl and 2-hydroxyethyl groups at positions 3, 4 and 5, respectively.		4.2730primary alcohol;
vitamin B1		Escherichia coli metabolite;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
uric acid
Uric Acid: An oxidation product, via XANTHINE OXIDASE, of oxypurines such as XANTHINE and HYPOXANTHINE. It is the final oxidation product of purine catabolism in humans and primates, whereas in most other mammals URATE OXIDASE further oxidizes it to ALLANTOIN.. uric acid : An oxopurine that is the final oxidation product of purine metabolism.. 6-hydroxy-1H-purine-2,8(7H,9H)-dione : A tautomer of uric acid having oxo groups at C-2 and C-8 and a hydroxy group at C-6.. 7,9-dihydro-1H-purine-2,6,8(3H)-trione : An oxopurine in which the purine ring is substituted by oxo groups at positions 2, 6, and 8.		3.2260uric acidEscherichia coli metabolite;
human metabolite;
mouse metabolite
urea
pseudourea: clinical use; structure. isourea : A carboximidic acid that is the imidic acid tautomer of urea, H2NC(=NH)OH, and its hydrocarbyl derivatives.		10.4180isourea;
monocarboxylic acid amide;
one-carbon compound		Daphnia magna metabolite;
Escherichia coli metabolite;
fertilizer;
flour treatment agent;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
2-amino-5-phosphonovalerate
2-Amino-5-phosphonovalerate: The D-enantiomer is a potent and specific antagonist of NMDA glutamate receptors (RECEPTORS, N-METHYL-D-ASPARTATE). The L form is inactive at NMDA receptors but may affect the AP4 (2-amino-4-phosphonobutyrate; APB) excitatory amino acid receptors.		210non-proteinogenic alpha-amino acidNMDA receptor antagonist
vanilmandelic acid
Vanilmandelic Acid: A 3-O-methyl ether of 3,4-dihydroxymandelic acid. It is an end-stage metabolite of CATECHOLAMINES; EPINEPHRINE; and NOREPINEPHRINE.. vanillylmandelic acid : An aromatic ether that is the 3-O-methyl ether of 3,4-dihydroxymandelic acid.		1.97102-hydroxy monocarboxylic acid;
aromatic ether;
phenols		human metabolite
meglutol
Meglutol: An antilipemic agent which lowers cholesterol, triglycerides, serum beta-lipoproteins and phospholipids. It acts by interfering with the enzymatic steps involved in the conversion of acetate to hydroxymethylglutaryl coenzyme A as well as inhibiting the activity of HYDROXYMETHYLGLUTARYL COA REDUCTASES which is the rate limiting enzyme in the biosynthesis of cholesterol.. 3-hydroxy-3-methylglutaric acid : A dicarboxylic acid that is glutaric acid in which one of the two hydrogens at position 3 is substituted by a hydroxy group, while the other is substituted by a methyl group. It has been found to accumulate in urine of patients suffering from HMG-CoA lyase (3-hydroxy-3-methylglutaryl-CoA lyase, EC 4.1.3.4) deficiency. It occurs as a plant metabolite in Crotalaria dura.		3.08503-hydroxy carboxylic acid;
dicarboxylic acid;
tertiary alcohol		anticholesteremic drug;
antimetabolite;
EC 1.1.1.34/EC 1.1.1.88 (hydroxymethylglutaryl-CoA reductase) inhibitor;
human metabolite;
plant metabolite
3-nitropropionic acid
3-nitropropionic acid: succinate dehydrogenase inactivator; biosynthesized by FABACEAE plants from ASPARAGINE. 3-nitropropanoic acid : A C-nitro compound that is propanoic acid in which one of the methyl hydrogens has been replaced by a nitro group.		2.7130C-nitro compoundantimycobacterial drug;
EC 1.3.5.1 [succinate dehydrogenase (quinone)] inhibitor;
mycotoxin;
neurotoxin
homovanillic acid
Homovanillic Acid: A 3-O-methyl ETHER of (3,4-dihydroxyphenyl)acetic acid.. homovanillate : A hydroxy monocarboxylic acid anion which is obtained by deprotonation of the carboxy group of homovanillic acid.. homovanillic acid : A monocarboxylic acid that is the 3-O-methyl ether of (3,4-dihydroxyphenyl)acetic acid. It is a catecholamine metabolite.		2.3720guaiacols;
monocarboxylic acid		human metabolite;
mouse metabolite
5-hydroxydecanoate
5-hydroxydecanoic acid: Potassium Channel Blocker; RN refers to parent cpd		2.0310medium-chain fatty acid
hydroxyindoleacetic acid
(5-hydroxyindol-3-yl)acetic acid : A member of the class of indole-3-acetic acids that is indole-3-acetic acid substituted by a hydroxy group at C-5.		1.9710indole-3-acetic acidsdrug metabolite;
human metabolite;
mouse metabolite
7-nitroindazole
7-nitroindazole: an inhibitor of nitric oxide synthase; exhibits anti-nociceptive activity without increasing blood pressure		2.0310
amprolium
Amprolium: A veterinary coccidiostat that interferes with THIAMINE metabolism.. amprolium : An organic chloride salt having 1-[(4-amino-2-propylpyrimidin-5-yl)methyl]-2-methylpyridin-1-ium as the counterion. Used for prevention of coccidiosis in poultry and cattle.. amprolium(1+) : A pyridinium ion that is the cationic portion of amprolium, a veterinary drug which is used for prevention of coccidiosis in poultry and cattle.		1.9510pyridinium ioncoccidiostat
baclofen
[no description available]		2.0310amino acid zwitterion;
gamma-amino acid;
monocarboxylic acid;
monochlorobenzenes;
primary amino compound		central nervous system depressant;
GABA agonist;
muscle relaxant
carmustine
Carmustine: A cell-cycle phase nonspecific alkylating antineoplastic agent. It is used in the treatment of brain tumors and various other malignant neoplasms. (From Martindale, The Extra Pharmacopoeia, 30th ed, p462) This substance may reasonably be anticipated to be a carcinogen according to the Fourth Annual Report on Carcinogens (NTP 85-002, 1985). (From Merck Index, 11th ed). carmustine : A member of the class of N-nitrosoureas that is 1,3-bis(2-chloroethyl)urea in which one of the nitrogens is substituted by a nitroso group.		2.0510N-nitrosoureas;
organochlorine compound		alkylating agent;
antineoplastic agent
celecoxib
[no description available]		2.0710organofluorine compound;
pyrazoles;
sulfonamide;
toluenes		cyclooxygenase 2 inhibitor;
geroprotector;
non-narcotic analgesic;
non-steroidal anti-inflammatory drug
cimetidine
Cimetidine: A histamine congener, it competitively inhibits HISTAMINE binding to HISTAMINE H2 RECEPTORS. Cimetidine has a range of pharmacological actions. It inhibits GASTRIC ACID secretion, as well as PEPSIN and GASTRIN output.. cimetidine : A member of the class of guanidines that consists of guanidine carrying a methyl substituent at position 1, a cyano group at position 2 and a 2-{[(5-methyl-1H-imidazol-4-yl)methyl]sulfanyl}ethyl group at position 3. It is a H2-receptor antagonist that inhibits the production of acid in stomach.		2.0110aliphatic sulfide;
guanidines;
imidazoles;
nitrile		adjuvant;
analgesic;
anti-ulcer drug;
H2-receptor antagonist;
P450 inhibitor
diazoxide
Diazoxide: A benzothiadiazine derivative that is a peripheral vasodilator used for hypertensive emergencies. It lacks diuretic effect, apparently because it lacks a sulfonamide group.. diazoxide : A benzothiadiazine that is the S,S-dioxide of 2H-1,2,4-benzothiadiazine which is substituted at position 3 by a methyl group and at position 7 by chlorine. A peripheral vasodilator, it increases the concentration of glucose in the plasma and inhibits the secretion of insulin by the beta- cells of the pancreas. It is used orally in the management of intractable hypoglycaemia and intravenously in the management of hypertensive emergencies.		2.0310benzothiadiazine;
organochlorine compound;
sulfone		antihypertensive agent;
beta-adrenergic agonist;
bronchodilator agent;
cardiotonic drug;
diuretic;
K-ATP channel agonist;
sodium channel blocker;
sympathomimetic agent;
vasodilator agent
pentetic acid
Pentetic Acid: An iron chelating agent with properties like EDETIC ACID. DTPA has also been used as a chelator for other metals, such as plutonium.		1.9510pentacarboxylic acidcopper chelator
dinitolmide
Dinitolmide: A coccidiostat for poultry.		1.9510dinitrotoluene
erythrosine
Fluoresceins: A family of spiro(isobenzofuran-1(3H),9'-(9H)xanthen)-3-one derivatives. These are used as dyes, as indicators for various metals, and as fluorescent labels in immunoassays.		2.4820
fenbendazole
Fenbendazole: Antinematodal benzimidazole used in veterinary medicine.. fenbendazole : A member of the class of benzimidazoles that is 1H-benzimidazole which is substituted at positons 2 and 5 by (methoxycarbonyl)amino and phenylsulfanediyl groups, respectively. A broad-spectrum anthelmintic, it is used, particularly in veterinary medicine, for the treatment of nematodal infections.		1.9710aryl sulfide;
benzimidazoles;
carbamate ester		antinematodal drug
fluorouracil
Fluorouracil: A pyrimidine analog that is an antineoplastic antimetabolite. It interferes with DNA synthesis by blocking the THYMIDYLATE SYNTHETASE conversion of deoxyuridylic acid to thymidylic acid.. 5-fluorouracil : A nucleobase analogue that is uracil in which the hydrogen at position 5 is replaced by fluorine. It is an antineoplastic agent which acts as an antimetabolite - following conversion to the active deoxynucleotide, it inhibits DNA synthesis (by blocking the conversion of deoxyuridylic acid to thymidylic acid by the cellular enzyme thymidylate synthetase) and so slows tumour growth.		2.0510nucleobase analogue;
organofluorine compound		antimetabolite;
antineoplastic agent;
environmental contaminant;
immunosuppressive agent;
radiosensitizing agent;
xenobiotic
gentamicin
Gentamicins: A complex of closely related aminoglycosides obtained from MICROMONOSPORA purpurea and related species. They are broad-spectrum antibiotics, but may cause ear and kidney damage. They act to inhibit PROTEIN BIOSYNTHESIS.		2.7830
hydroxyurea
[no description available]		2.0510one-carbon compound;
ureas		antimetabolite;
antimitotic;
antineoplastic agent;
DNA synthesis inhibitor;
EC 1.17.4.1 (ribonucleoside-diphosphate reductase) inhibitor;
genotoxin;
immunomodulator;
radical scavenger;
teratogenic agent
ifenprodil
ifenprodil: NMDA receptor antagonist		210piperidines
isoflurane
Isoflurane: A stable, non-explosive inhalation anesthetic, relatively free from significant side effects.		2.4220organofluorine compoundinhalation anaesthetic
2-amino-3-phosphonopropionic acid
2-amino-3-phosphonopropionic acid: metabotropic glutamate receptor antagonist; do not confuse AP-3 used as an abbreviation for this with enhancer-binding protein AP-3 (a trans-activator) or clathrin assembly protein AP-3. 2-amino-3-phosphonopropanoic acid : A non-proteinogenc alpha-amino acid that is alanine in which one of the hydrogens of the terminal methyl group has been replaced by a dihydroxy(oxido)-lambda(5)-phosphanyl group.		210alanine derivative;
non-proteinogenic alpha-amino acid;
phosphonic acids		human metabolite;
metabotropic glutamate receptor antagonist
metformin
Metformin: A biguanide hypoglycemic agent used in the treatment of non-insulin-dependent diabetes mellitus not responding to dietary modification. Metformin improves glycemic control by improving insulin sensitivity and decreasing intestinal absorption of glucose. (From Martindale, The Extra Pharmacopoeia, 30th ed, p289). metformin : A member of the class of guanidines that is biguanide the carrying two methyl substituents at position 1.		7.4572guanidinesenvironmental contaminant;
geroprotector;
hypoglycemic agent;
xenobiotic
methoxyamine
methoxyamine: analytical reagent for aldehydes and ketones; strong irritant, can probably produce methemoglobinemia; RN given refers to parent cpd; structure		2.0210organooxygen compound
metoclopramide
Metoclopramide: A dopamine D2 antagonist that is used as an antiemetic.. metoclopramide : A member of the class of benzamides resulting from the formal condensation of 4-amino-5-chloro-2-methoxybenzoic acid with the primary amino group of N,N-diethylethane-1,2-diamine.		7.0110benzamides;
monochlorobenzenes;
substituted aniline;
tertiary amino compound		antiemetic;
dopaminergic antagonist;
environmental contaminant;
gastrointestinal drug;
xenobiotic
metronidazole
Metronidazole: A nitroimidazole used to treat AMEBIASIS; VAGINITIS; TRICHOMONAS INFECTIONS; GIARDIASIS; ANAEROBIC BACTERIA; and TREPONEMAL INFECTIONS.. metronidazole : A member of the class of imidazoles substituted at C-1, -2 and -5 with 2-hydroxyethyl, nitro and methyl groups respectively. It has activity against anaerobic bacteria and protozoa, and has a radiosensitising effect on hypoxic tumour cells. It may be given by mouth in tablets, or as the benzoate in an oral suspension. The hydrochloride salt can be used in intravenous infusions. Metronidazole is a prodrug and is selective for anaerobic bacteria due to their ability to intracellularly reduce the nitro group of metronidazole to give nitroso-containing intermediates. These can covalently bind to DNA, disrupting its helical structure, inducing DNA strand breaks and inhibiting bacterial nucleic acid synthesis, ultimately resulting in bacterial cell death.		10.0252C-nitro compound;
imidazoles;
primary alcohol		antiamoebic agent;
antibacterial drug;
antimicrobial agent;
antiparasitic agent;
antitrichomonal drug;
environmental contaminant;
prodrug;
radiosensitizing agent;
xenobiotic
monodansylcadaverine
monodansylcadaverine: inhibits cross linkage of fibrin. monodansylcadaverine : A sulfonamide obtained by formal condensation of the sulfo group of 5-(dimethylamino)naphthalene-1-sulfonic acid with one of the amino groups of cadaverine.		1.9810aminonaphthalene;
primary amino compound;
sulfonamide;
tertiary amino compound		EC 2.3.2.13 (protein-glutamine gamma-glutamyltransferase) inhibitor;
fluorochrome;
protective agent
muscimol
Muscimol: A neurotoxic isoxazole isolated from species of AMANITA. It is obtained by decarboxylation of IBOTENIC ACID. Muscimol is a potent agonist of GABA-A RECEPTORS and is used mainly as an experimental tool in animal and tissue studies.. muscimol : A member of the class of isoxazoles that is 1,2-oxazol-3(2H)-one substituted by an aminomethyl group at position 5. It has been isolated from mushrooms of the genus Amanita.		2.0310alkaloid;
isoxazoles;
primary amino compound		fungal metabolite;
GABA agonist;
oneirogen;
psychotropic drug
omeprazole
Omeprazole: A 4-methoxy-3,5-dimethylpyridyl, 5-methoxybenzimidazole derivative of timoprazole that is used in the therapy of STOMACH ULCERS and ZOLLINGER-ELLISON SYNDROME. The drug inhibits an H(+)-K(+)-EXCHANGING ATPASE which is found in GASTRIC PARIETAL CELLS.. omeprazole : A racemate comprising equimolar amounts of (R)- and (S)-omeprazole.. 5-methoxy-2-{[(4-methoxy-3,5-dimethylpyridin-2-yl)methyl]sulfinyl}-1H-benzimidazole : A member of the class of benzimidazoles that is 1H-benzimidazole which is substituted by a [4-methoxy-3,5-dimethylpyridin-2-yl)methyl]sulfinyl group at position 2 and a methoxy group at position 5.		2.0110aromatic ether;
benzimidazoles;
pyridines;
sulfoxide
pentoxifylline
[no description available]		2.0710oxopurine
propranolol
Propranolol: A widely used non-cardioselective beta-adrenergic antagonist. Propranolol has been used for MYOCARDIAL INFARCTION; ARRHYTHMIA; ANGINA PECTORIS; HYPERTENSION; HYPERTHYROIDISM; MIGRAINE; PHEOCHROMOCYTOMA; and ANXIETY but adverse effects instigate replacement by newer drugs.. propranolol : A propanolamine that is propan-2-ol substituted by a propan-2-ylamino group at position 1 and a naphthalen-1-yloxy group at position 3.		2.730naphthalenes;
propanolamine;
secondary amine		anti-arrhythmia drug;
antihypertensive agent;
anxiolytic drug;
beta-adrenergic antagonist;
environmental contaminant;
human blood serum metabolite;
vasodilator agent;
xenobiotic
sebacic acid
sebacic acid : An alpha,omega-dicarboxylic acid that is the 1,8-dicarboxy derivative of octane.		210alpha,omega-dicarboxylic acid;
dicarboxylic fatty acid		human metabolite;
plant metabolite
sodium fluoride
[no description available]		1.9910fluoride saltmutagen
temozolomide
[no description available]		2.110imidazotetrazine;
monocarboxylic acid amide;
triazene derivative		alkylating agent;
antineoplastic agent;
prodrug
sorbitol
D-glucitol : The D-enantiomer of glucitol (also known as D-sorbitol).		1.9710glucitolcathartic;
Escherichia coli metabolite;
food humectant;
human metabolite;
laxative;
metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite;
sweetening agent
thymidine
[no description available]		9.2741pyrimidine 2'-deoxyribonucleosideEscherichia coli metabolite;
human metabolite;
metabolite;
mouse metabolite
aldosterone
[no description available]		1.971011beta-hydroxy steroid;
18-oxo steroid;
20-oxo steroid;
21-hydroxy steroid;
3-oxo-Delta(4) steroid;
C21-steroid hormone;
mineralocorticoid;
primary alpha-hydroxy ketone;
steroid aldehyde		human metabolite;
mouse metabolite
prednisone
Prednisone: A synthetic anti-inflammatory glucocorticoid derived from CORTISONE. It is biologically inert and converted to PREDNISOLONE in the liver.. prednisone : A synthetic glucocorticoid drug that is particularly effective as an immunosuppressant, and affects virtually all of the immune system. Prednisone is a prodrug that is converted by the liver into prednisolone (a beta-hydroxy group instead of the oxo group at position 11), which is the active drug and also a steroid.		2.021011-oxo steroid;
17alpha-hydroxy steroid;
20-oxo steroid;
21-hydroxy steroid;
3-oxo-Delta(1),Delta(4)-steroid;
C21-steroid;
glucocorticoid;
primary alpha-hydroxy ketone;
tertiary alpha-hydroxy ketone		adrenergic agent;
anti-inflammatory drug;
antineoplastic agent;
immunosuppressive agent;
prodrug
alanine
Alanine: A non-essential amino acid that occurs in high levels in its free state in plasma. It is produced from pyruvate by transamination. It is involved in sugar and acid metabolism, increases IMMUNITY, and provides energy for muscle tissue, BRAIN, and the CENTRAL NERVOUS SYSTEM.. alanine : An alpha-amino acid that consists of propionic acid bearing an amino substituent at position 2.		3.2360alanine zwitterion;
alanine;
L-alpha-amino acid;
proteinogenic amino acid;
pyruvate family amino acid		EC 4.3.1.15 (diaminopropionate ammonia-lyase) inhibitor;
fundamental metabolite
serine
Serine: A non-essential amino acid occurring in natural form as the L-isomer. It is synthesized from GLYCINE or THREONINE. It is involved in the biosynthesis of PURINES; PYRIMIDINES; and other amino acids.. serine : An alpha-amino acid that is alanine substituted at position 3 by a hydroxy group.		2.4220L-alpha-amino acid;
proteinogenic amino acid;
serine family amino acid;
serine zwitterion;
serine		algal metabolite;
Escherichia coli metabolite;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
aspartic acid
Aspartic Acid: One of the non-essential amino acids commonly occurring in the L-form. It is found in animals and plants, especially in sugar cane and sugar beets. It may be a neurotransmitter.. aspartic acid : An alpha-amino acid that consists of succinic acid bearing a single alpha-amino substituent. L-aspartic acid : The L-enantiomer of aspartic acid.		1.9910aspartate family amino acid;
aspartic acid;
L-alpha-amino acid;
proteinogenic amino acid		Escherichia coli metabolite;
mouse metabolite;
neurotransmitter
glutamine
Glutamine: A non-essential amino acid present abundantly throughout the body and is involved in many metabolic processes. It is synthesized from GLUTAMIC ACID and AMMONIA. It is the principal carrier of NITROGEN in the body and is an important energy source for many cells.. L-glutamine : An optically active form of glutamine having L-configuration.. glutamine : An alpha-amino acid that consists of butyric acid bearing an amino substituent at position 2 and a carbamoyl substituent at position 4.		2.1310amino acid zwitterion;
glutamine family amino acid;
glutamine;
L-alpha-amino acid;
polar amino acid zwitterion;
proteinogenic amino acid		EC 1.14.13.39 (nitric oxide synthase) inhibitor;
Escherichia coli metabolite;
human metabolite;
metabolite;
micronutrient;
mouse metabolite;
nutraceutical;
Saccharomyces cerevisiae metabolite
lysine
Lysine: An essential amino acid. It is often added to animal feed.. lysine : A diamino acid that is caproic (hexanoic) acid bearing two amino substituents at positions 2 and 6.. L-lysine : An L-alpha-amino acid; the L-isomer of lysine.		9.3360aspartate family amino acid;
L-alpha-amino acid zwitterion;
L-alpha-amino acid;
lysine;
organic molecular entity;
proteinogenic amino acid		algal metabolite;
anticonvulsant;
Escherichia coli metabolite;
human metabolite;
micronutrient;
mouse metabolite;
nutraceutical;
plant metabolite;
Saccharomyces cerevisiae metabolite
adenosine diphosphate
Adenosine Diphosphate: Adenosine 5'-(trihydrogen diphosphate). An adenine nucleotide containing two phosphate groups esterified to the sugar moiety at the 5'-position.		2.3920adenosine 5'-phosphate;
purine ribonucleoside 5'-diphosphate		fundamental metabolite;
human metabolite
bromodeoxyuridine
Bromodeoxyuridine: A nucleoside that substitutes for thymidine in DNA and thus acts as an antimetabolite. It causes breaks in chromosomes and has been proposed as an antiviral and antineoplastic agent. It has been given orphan drug status for use in the treatment of primary brain tumors.		2.0110pyrimidine 2'-deoxyribonucleosideantimetabolite;
antineoplastic agent
galactose
galactopyranose : The pyranose form of galactose.		1.9510D-galactose;
galactopyranose		Escherichia coli metabolite;
mouse metabolite
levodopa
Levodopa: The naturally occurring form of DIHYDROXYPHENYLALANINE and the immediate precursor of DOPAMINE. Unlike dopamine itself, it can be taken orally and crosses the blood-brain barrier. It is rapidly taken up by dopaminergic neurons and converted to DOPAMINE. It is used for the treatment of PARKINSONIAN DISORDERS and is usually given with agents that inhibit its conversion to dopamine outside of the central nervous system.. L-dopa : An optically active form of dopa having L-configuration. Used to treat the stiffness, tremors, spasms, and poor muscle control of Parkinson's disease		10.1650amino acid zwitterion;
dopa;
L-tyrosine derivative;
non-proteinogenic L-alpha-amino acid		allelochemical;
antidyskinesia agent;
antiparkinson drug;
dopaminergic agent;
hapten;
human metabolite;
mouse metabolite;
neurotoxin;
plant growth retardant;
plant metabolite;
prodrug
edetic acid
Edetic Acid: A chelating agent that sequesters a variety of polyvalent cations such as CALCIUM. It is used in pharmaceutical manufacturing and as a food additive.		2.3720ethylenediamine derivative;
polyamino carboxylic acid;
tetracarboxylic acid		anticoagulant;
antidote;
chelator;
copper chelator;
geroprotector
tyrosine
Tyrosine: A non-essential amino acid. In animals it is synthesized from PHENYLALANINE. It is also the precursor of EPINEPHRINE; THYROID HORMONES; and melanin.. tyrosine : An alpha-amino acid that is phenylalanine bearing a hydroxy substituent at position 4 on the phenyl ring.		4.9891amino acid zwitterion;
erythrose 4-phosphate/phosphoenolpyruvate family amino acid;
L-alpha-amino acid;
proteinogenic amino acid;
tyrosine		EC 1.3.1.43 (arogenate dehydrogenase) inhibitor;
fundamental metabolite;
micronutrient;
nutraceutical
methylene blue
Methylene Blue: A compound consisting of dark green crystals or crystalline powder, having a bronze-like luster. Solutions in water or alcohol have a deep blue color. Methylene blue is used as a bacteriologic stain and as an indicator. It inhibits GUANYLATE CYCLASE, and has been used to treat cyanide poisoning and to lower levels of METHEMOGLOBIN.. methylene blue : An organic chloride salt having 3,7-bis(dimethylamino)phenothiazin-5-ium as the counterion. A commonly used dye that also exhibits antioxidant, antimalarial, antidepressant and cardioprotective properties.		2.0310organic chloride saltacid-base indicator;
antidepressant;
antimalarial;
antimicrobial agent;
antioxidant;
cardioprotective agent;
EC 1.4.3.4 (monoamine oxidase) inhibitor;
EC 3.1.1.8 (cholinesterase) inhibitor;
EC 4.6.1.2 (guanylate cyclase) inhibitor;
fluorochrome;
histological dye;
neuroprotective agent;
physical tracer
leucine
Leucine: An essential branched-chain amino acid important for hemoglobin formation.. leucine : A branched-chain amino acid that consists of glycine in which one of the hydrogens attached to the alpha-carbon is substituted by an isobutyl group.		5.0880amino acid zwitterion;
L-alpha-amino acid;
leucine;
proteinogenic amino acid;
pyruvate family amino acid		algal metabolite;
Escherichia coli metabolite;
human metabolite;
mouse metabolite;
plant metabolite;
Saccharomyces cerevisiae metabolite
2-aminoisobutyric acid
2-aminoisobutyric acid: RN given refers to unlabeled cpd. 2-aminoisobutyric acid : A rare, non-protein amino acid and end-product of pyrimidine metabolism, excreted in urine and found in some antibiotics of fungal origin. With the exception of a few bacteria, it is non-metabolisable, and therefore used in bioassays.		1.98102,2-dialkylglycine zwitterion;
2,2-dialkylglycine
methionine
Methionine: A sulfur-containing essential L-amino acid that is important in many body functions.. methionine : A sulfur-containing amino acid that is butyric acid bearing an amino substituent at position 2 and a methylthio substituent at position 4.		10.18411aspartate family amino acid;
L-alpha-amino acid;
methionine zwitterion;
methionine;
proteinogenic amino acid		antidote to paracetamol poisoning;
human metabolite;
micronutrient;
mouse metabolite;
nutraceutical
1,2-dipalmitoylphosphatidylcholine
1,2-Dipalmitoylphosphatidylcholine: Synthetic phospholipid used in liposomes and lipid bilayers to study biological membranes. It is also a major constituent of PULMONARY SURFACTANTS.		2.0510
phenylalanine
Phenylalanine: An essential aromatic amino acid that is a precursor of MELANIN; DOPAMINE; noradrenalin (NOREPINEPHRINE), and THYROXINE.. L-phenylalanine : The L-enantiomer of phenylalanine.. phenylalanine : An aromatic amino acid that is alanine in which one of the methyl hydrogens is substituted by a phenyl group.		3.2460amino acid zwitterion;
erythrose 4-phosphate/phosphoenolpyruvate family amino acid;
L-alpha-amino acid;
phenylalanine;
proteinogenic amino acid		algal metabolite;
EC 3.1.3.1 (alkaline phosphatase) inhibitor;
Escherichia coli metabolite;
human xenobiotic metabolite;
micronutrient;
mouse metabolite;
nutraceutical;
plant metabolite;
Saccharomyces cerevisiae metabolite
dimethylformamide
Dimethylformamide: A formamide in which the amino hydrogens are replaced by methyl groups.. N,N-dimethylformamide : A member of the class of formamides that is formamide in which the amino hydrogens are replaced by methyl groups.		1.9810formamides;
volatile organic compound		geroprotector;
hepatotoxic agent;
polar aprotic solvent
ampicillin
Ampicillin: Semi-synthetic derivative of penicillin that functions as an orally active broad-spectrum antibiotic.. ampicillin : A penicillin in which the substituent at position 6 of the penam ring is a 2-amino-2-phenylacetamido group.		1.9510beta-lactam antibiotic;
penicillin allergen;
penicillin		antibacterial drug
asparagine
Asparagine: A non-essential amino acid that is involved in the metabolic control of cell functions in nerve and brain tissue. It is biosynthesized from ASPARTIC ACID and AMMONIA by asparagine synthetase. (From Concise Encyclopedia Biochemistry and Molecular Biology, 3rd ed). asparagine : An alpha-amino acid in which one of the hydrogens attached to the alpha-carbon of glycine is substituted by a 2-amino-2-oxoethyl group.		210amino acid zwitterion;
asparagine;
aspartate family amino acid;
L-alpha-amino acid;
proteinogenic amino acid		Escherichia coli metabolite;
human metabolite;
micronutrient;
mouse metabolite;
nutraceutical;
plant metabolite;
Saccharomyces cerevisiae metabolite
histidine
Histidine: An essential amino acid that is required for the production of HISTAMINE.. L-histidine : The L-enantiomer of the amino acid histidine.. histidine : An alpha-amino acid that is propanoic acid bearing an amino substituent at position 2 and a 1H-imidazol-4-yl group at position 3.		3.5790amino acid zwitterion;
histidine;
L-alpha-amino acid;
polar amino acid zwitterion;
proteinogenic amino acid		algal metabolite;
Escherichia coli metabolite;
human metabolite;
micronutrient;
mouse metabolite;
nutraceutical;
Saccharomyces cerevisiae metabolite
valine
Valine: A branched-chain essential amino acid that has stimulant activity. It promotes muscle growth and tissue repair. It is a precursor in the penicillin biosynthetic pathway.. valine : A branched-chain amino acid that consists of glycine in which one of the hydrogens attached to the alpha-carbon is substituted by an isopropyl group.. L-valine : The L-enantiomer of valine.		5.97210L-alpha-amino acid zwitterion;
L-alpha-amino acid;
proteinogenic amino acid;
pyruvate family amino acid;
valine		algal metabolite;
Escherichia coli metabolite;
human metabolite;
micronutrient;
mouse metabolite;
nutraceutical;
Saccharomyces cerevisiae metabolite
threonine
Threonine: An essential amino acid occurring naturally in the L-form, which is the active form. It is found in eggs, milk, gelatin, and other proteins.. threonine : An alpha-amino acid in which one of the hydrogens attached to the alpha-carbon of glycine is substituted by a 1-hydroxyethyl group.		4.0731amino acid zwitterion;
aspartate family amino acid;
L-alpha-amino acid;
proteinogenic amino acid;
threonine		algal metabolite;
Escherichia coli metabolite;
human metabolite;
micronutrient;
mouse metabolite;
nutraceutical;
plant metabolite;
Saccharomyces cerevisiae metabolite
isoleucine
Isoleucine: An essential branched-chain aliphatic amino acid found in many proteins. It is an isomer of LEUCINE. It is important in hemoglobin synthesis and regulation of blood sugar and energy levels.. isoleucine : A 2-amino-3-methylpentanoic acid having either (2R,3R)- or (2S,3S)-configuration.. L-isoleucine : The L-enantiomer of isoleucine.		5.78170aspartate family amino acid;
isoleucine;
L-alpha-amino acid zwitterion;
L-alpha-amino acid;
proteinogenic amino acid		algal metabolite;
Escherichia coli metabolite;
human metabolite;
mouse metabolite;
plant metabolite;
Saccharomyces cerevisiae metabolite
arginine
Arginine: An essential amino acid that is physiologically active in the L-form.. arginine : An alpha-amino acid that is glycine in which the alpha-is substituted by a 3-guanidinopropyl group.		3.0650arginine;
glutamine family amino acid;
L-alpha-amino acid;
proteinogenic amino acid		biomarker;
Escherichia coli metabolite;
micronutrient;
mouse metabolite;
nutraceutical
trichloroacetic acid
Trichloroacetic Acid: A strong acid used as a protein precipitant in clinical chemistry and also as a caustic for removing warts.. trichloroacetic acid : A monocarboxylic acid that is acetic acid in which all three methyl hydrogens are substituted by chlorine.		7.610monocarboxylic acid;
organochlorine compound		carcinogenic agent;
metabolite;
mouse metabolite
trifluoroacetic acid
Trifluoroacetic Acid: A very strong halogenated derivative of acetic acid. It is used in acid catalyzed reactions, especially those where an ester is cleaved in peptide synthesis.. trifluoroacetic acid : A monocarboxylic acid that is the trifluoro derivative of acetic acid.		2.0210fluoroalkanoic acidhuman xenobiotic metabolite;
NMR chemical shift reference compound;
reagent
trichloroethylene
Trichloroethylene: A highly volatile inhalation anesthetic used mainly in short surgical procedures where light anesthesia with good analgesia is required. It is also used as an industrial solvent. Prolonged exposure to high concentrations of the vapor can lead to cardiotoxicity and neurological impairment.. triol : A chemical compound containing three hydroxy groups.		2.4520chloroethenesinhalation anaesthetic;
mouse metabolite
methyl chloroformate
[no description available]		2.0210
isobutyric acid
isobutyric acid: RN given refers to parent cpd. isobutyric acid : A branched fatty acid comprising propanoic acid carrying a methyl branch at C-2.		1.9510branched-chain saturated fatty acid;
fatty acid 4:0;
methyl-branched fatty acid		Daphnia magna metabolite;
plant metabolite;
volatile oil component
methylmethacrylate
Methylmethacrylate: The methyl ester of methacrylic acid. It polymerizes easily to form POLYMETHYL METHACRYLATE. It is used as a bone cement.. methyl methacrylate : An enoate ester having methacrylic acid as the carboxylic acid component and methanol as the alcohol component.		1.9610enoate ester;
methyl ester		allergen;
polymerisation monomer
pyridoxic acid
Pyridoxic Acid: The catabolic product of most of VITAMIN B 6; (PYRIDOXINE; PYRIDOXAL; and PYRIDOXAMINE) which is excreted in the urine.. 4-pyridoxic acid : A methylpyridine that is 2-methylpyridine substituted by a hydroxy group at C-3, a carboxy group at C-4, and a hydroxymethyl group at C-5. It is the catabolic product of vitamin B6 and is excreted in the urine.. 4-pyridoxate : A pyridoxate that is the conjugate base of 4-pyridoxic acid, obtained by deprotonation of the carboxy group.		2.0110hydroxymethylpyridine;
methylpyridines;
monohydroxypyridine;
vitamin B6		human urinary metabolite;
mouse metabolite
rotenone
Derris: A plant genus of the family FABACEAE. The root is a source of rotenoids (ROTENONE) and flavonoids. Some species of Pongamia have been reclassified to this genus and some to MILLETTIA. Some species of Deguelia have been reclassified to this genus.. rotenoid : Members of the class of tetrahydrochromenochromene that consists of a cis-fused tetrahydrochromeno[3,4-b]chromene skeleton and its substituted derivatives. The term was originally restricted to natural products, but is now also used to describe semi-synthetic and fully synthetic compounds.		2.0210organic heteropentacyclic compound;
rotenones		antineoplastic agent;
metabolite;
mitochondrial NADH:ubiquinone reductase inhibitor;
phytogenic insecticide;
piscicide;
toxin
xylitol
xylooligosaccharide: structure in first source. pentitol : An alditol obtained by reduction of any pentose.. xylooligosaccharide : An oligosaccharide comprised of xylose residues.		1.9710
isatin
tribulin: endogenous MONOAMINE OXIDASE inhibitory activity extractable into ethyl acetate found in brain and many mammalian tissues and fluids; ISATIN is a major component; produced in excess following alcohol withdrawal;		2.1310indoledioneEC 1.4.3.4 (monoamine oxidase) inhibitor;
plant metabolite
methyl nicotinate
methyl nicotinate: erythema provoked is basis of a methylnicotinate test of anti-inflammatories		2.1310aromatic carboxylic acid;
pyridines
propylparaben
Parabens: Methyl, propyl, butyl, and ethyl esters of p-hydroxybenzoic acid. They have been approved by the FDA as antimicrobial agents for foods and pharmaceuticals. (From Hawley's Condensed Chemical Dictionary, 11th ed, p872)		210benzoate ester;
paraben;
phenols		antifungal agent;
antimicrobial agent
pyrrolidonecarboxylic acid
Pyrrolidonecarboxylic Acid: A cyclized derivative of L-GLUTAMIC ACID. Elevated blood levels may be associated with problems of GLUTAMINE or GLUTATHIONE metabolism.. 5-oxo-L-proline : An optically active form of 5-oxoproline having L-configuration.		1.99105-oxoproline;
L-proline derivative;
non-proteinogenic L-alpha-amino acid		algal metabolite
trehalose
alpha,alpha-trehalose : A trehalose in which both glucose residues have alpha-configuration at the anomeric carbon.		210trehaloseEscherichia coli metabolite;
geroprotector;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
4-anisic acid
4-methoxybenzoic acid: structure in first source. 4-methoxybenzoic acid : A methoxybenzoic acid substituted with a methoxy group at position C-4.		210methoxybenzoic acidplant metabolite
methyl malonate
methyl malonate: do not confuse with methylmalonate, i.e., malonic acid substituted with a methyl group on C2; structure		2.1110
pyrroles
1H-pyrrole : A tautomer of pyrrole that has the double bonds at positions 2 and 4.. pyrrole : A five-membered monocyclic heteroarene comprising one NH and four CH units which forms the parent compound of the pyrrole group of compounds. Its five-membered ring structure has three tautomers. A 'closed class'.. azole : Any monocyclic heteroarene consisting of a five-membered ring containing nitrogen. Azoles can also contain one or more other non-carbon atoms, such as nitrogen, sulfur or oxygen.		2.2510pyrrole;
secondary amine
2,2,2-trichloroethanol
[no description available]		2.4520chloroethanolmouse metabolite
framycetin
Framycetin: A component of NEOMYCIN that is produced by Streptomyces fradiae. On hydrolysis it yields neamine and neobiosamine B. (From Merck Index, 11th ed). framycetin : A tetracyclic antibacterial agent derived from neomycin, being a glycoside ester of neamine and neobiosamine B.		1.9710aminoglycosideallergen;
antibacterial drug;
Escherichia coli metabolite
indazoles
Indazoles: A group of heterocyclic aromatic organic compounds consisting of the fusion of BENZENE and PYRAZOLES.		2.0310indazole
azulene
[no description available]		210azulenes;
mancude carbobicyclic parent;
ortho-fused bicyclic arene		plant metabolite;
volatile oil component
thiazoles
[no description available]		2.02101,3-thiazoles;
mancude organic heteromonocyclic parent;
monocyclic heteroarene
pyrimidine
pyrimidine : The parent compound of the pyrimidines; a diazine having the two nitrogens at the 1- and 3-positions.		6.9310diazine;
pyrimidines		Daphnia magna metabolite
4-hydroxyphenyllactic acid
4-hydroxyphenyllactic acid: tyrosing metabolite; RN given refers to cpd without isomeric designation. 3-(4-hydroxyphenyl)lactic acid : A 2-hydroxy carboxylic acid that is lactic acid in which one of the methyl hydrogens is substituted by a 4-hydroxyphenyl group.		2.11102-hydroxy carboxylic acid;
phenols		bacterial metabolite;
human metabolite
nicarbazin
Nicarbazin: An equimolar complex of 4,4'-Dinitrocarbanilide and 2-Hydroxy-4,6-dimethylpyrimidine. A coccidiostat for poultry.		1.9510organic molecular entity
citrulline
citrulline : The parent compound of the citrulline class consisting of ornithine having a carbamoyl group at the N(5)-position.		2.6730amino acid zwitterion;
citrulline		Daphnia magna metabolite;
EC 1.14.13.39 (nitric oxide synthase) inhibitor;
Escherichia coli metabolite;
human metabolite;
micronutrient;
mouse metabolite;
nutraceutical;
plant metabolite;
protective agent;
Saccharomyces cerevisiae metabolite
4-fluorobenzoic acid
[no description available]		1.9810fluorobenzoic acidbacterial xenobiotic metabolite
fluorobenzenes
Fluorobenzenes: Derivatives of BENZENE that contain FLUORINE.. monofluorobenzene : The simplest member of the class of monofluorobenzenes that is benzene carrying a single fluoro substituent.. fluorobenzenes : Any fluoroarene that is a benzene or a substituted benzene carrying at least one fluoro group.		2.0410monofluorobenzenesNMR chemical shift reference compound
homocystine
[no description available]		4.981amino acid zwitterion;
homocystines		human metabolite
limestone
Calcium Carbonate: Carbonic acid calcium salt (CaCO3). An odorless, tasteless powder or crystal that occurs in nature. It is used therapeutically as a phosphate buffer in hemodialysis patients and as a calcium supplement.. calcium carbonate : A calcium salt with formula CCaO3.		3.5311calcium salt;
carbonate salt;
inorganic calcium salt;
one-carbon compound		antacid;
fertilizer;
food colouring;
food firming agent
isovaleric acid
isovaleric acid: structure. isovaleric acid : A C5, branched-chain saturated fatty acid.		10.09140branched-chain saturated fatty acid;
methylbutyric acid;
short-chain fatty acid		mammalian metabolite;
plant metabolite
suberic acid
suberic acid: used in plastics industry; inhibits activity of some enzymes; RN given refers to parent cpd; structure. suberic acid : An alpha,omega-dicarboxylic acid that is the 1,6-dicarboxy derivative of hexane.		210alpha,omega-dicarboxylic acid;
dicarboxylic fatty acid		human metabolite
2-n-butylmalonate
2-n-butylmalonate: structure		2.0710
dicyclohexylcarbodiimide
1,3-dicyclohexylcarbodiimide : A carbodiimide compound having a cyclohexyl substituent on both nitrogen atoms.		1.9610carbodiimideATP synthase inhibitor;
cross-linking reagent;
peptide coupling reagent
myristic acid
Myristic Acid: A saturated 14-carbon fatty acid occurring in most animal and vegetable fats, particularly butterfat and coconut, palm, and nutmeg oils. It is used to synthesize flavor and as an ingredient in soaps and cosmetics. (From Dorland, 28th ed). tetradecanoic acid : A straight-chain, fourteen-carbon, long-chain saturated fatty acid mostly found in milk fat.. tetradecanoate : A long-chain fatty acid anion that is the conjugate base of myristic acid; major species at pH 7.3.		1.9910long-chain fatty acid;
straight-chain saturated fatty acid		algal metabolite;
Daphnia magna metabolite;
EC 3.1.1.1 (carboxylesterase) inhibitor;
human metabolite
2-hydroxybutyric acid
2-hydroxybutyric acid: RN given refers to cpd without isomeric designation. hydroxybutyric acid : Any compound comprising a butyric acid core carrying at least one hydroxy substituent.. 2-hydroxybutyric acid : A hydroxybutyric acid having a single hydroxyl group located at position 2; urinary secretion of 2-hydroxybutyric acid is increased with alcohol ingestion or vigorous physical exercise and is associated with lactic acidosis and ketoacidosis in humans and diabetes in animals.		2.11102-hydroxy monocarboxylic acid;
hydroxybutyric acid		algal metabolite;
human metabolite
2,2-dimethylmalonate
dimethylmalonic acid : A dicarboxylic acid that is malonic acid in which both methylene hydrogens have been replaced by methyl groups.		2.1110dicarboxylic acidfatty acid synthesis inhibitor
ethylmalonic acid
ethylmalonic acid: don't confuse with diethyl malonate, which is a diester. ethylmalonate : A dicarboxylic acid anion obtained by deprotonation of at least one of the carboxy groups of ethylmalonic acid.. ethylmalonic acid : A dicarboxylic acid obtained by substitution of one of the methylene hydrogens of malonic acid by an ethyl group.		8.1350dicarboxylic acid;
dicarboxylic fatty acid		human metabolite
glycine ethyl ester
glycine ethyl ester: RN given refers to parent cpd		1.9610
erythromycin
Erythromycin: A bacteriostatic antibiotic macrolide produced by Streptomyces erythreus. Erythromycin A is considered its major active component. In sensitive organisms, it inhibits protein synthesis by binding to 50S ribosomal subunits. This binding process inhibits peptidyl transferase activity and interferes with translocation of amino acids during translation and assembly of proteins.. erythromycin : Any of several wide-spectrum macrolide antibiotics obtained from actinomycete Saccharopolyspora erythraea (formerly known as Streptomyces erythraeus).. erythromycin A : An erythromycin that consists of erythronolide A having 2,6-dideoxy-3-C-methyl-3-O-methyl-alpha-L-ribo-hexopyranosyl and 3,4,6-trideoxy-3-(dimethylamino)-beta-D-xylo-hexopyranosyl residues attahced at positions 4 and 6 respectively.		2.4220cyclic ketone;
erythromycin
deoxyuridine
[no description available]		6.81112pyrimidine 2'-deoxyribonucleosideEscherichia coli metabolite;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
d-alpha tocopherol
Vitamin E: A generic descriptor for all TOCOPHEROLS and TOCOTRIENOLS that exhibit ALPHA-TOCOPHEROL activity. By virtue of the phenolic hydrogen on the 2H-1-benzopyran-6-ol nucleus, these compounds exhibit varying degree of antioxidant activity, depending on the site and number of methyl groups and the type of ISOPRENOIDS.. tocopherol : A collective name for a group of closely related lipids that contain a chroman-6-ol nucleus substituted at position 2 by a methyl group and by a saturated hydrocarbon chain consisting of three isoprenoid units. They are designated as alpha-, beta-, gamma-, and delta-tocopherol depending on the number and position of additional methyl substituents on the aromatic ring. Tocopherols occur in vegetable oils and vegetable oil products, almost exclusively with R,R,R configuration. Tocotrienols differ from tocopherols only in having three double bonds in the hydrocarbon chain.. vitamin E : Any member of a group of fat-soluble chromanols that exhibit biological activity against vitamin E deficiency. The vitamers in this class consists of a chroman-6-ol core which is substituted at position 2 by a methyl group and (also at position 2) either a saturated or a triply-unsaturated hydrocarbon chain consisting of three isoprenoid units. The major function of vitamin E is to act as a natural antioxidant by scavenging free radicals and molecular oxygen.. (R,R,R)-alpha-tocopherol : An alpha-tocopherol that has R,R,R configuration. The naturally occurring stereoisomer of alpha-tocopherol, it is found particularly in sunflower and olive oils.		2.3720alpha-tocopherolalgal metabolite;
antiatherogenic agent;
anticoagulant;
antioxidant;
antiviral agent;
EC 2.7.11.13 (protein kinase C) inhibitor;
immunomodulator;
micronutrient;
nutraceutical;
plant metabolite
ethyldimethylaminopropyl carbodiimide
Ethyldimethylaminopropyl Carbodiimide: Carbodiimide cross-linking reagent.		1.9610
dithiothreitol
1,4-dimercaptobutane-2,3-diol : A glycol that is butane-2,3-diol in which a hydrogen from each of the methyl groups is replaced by a thiol group.. 1,4-dithiothreitol : The threo-diastereomer of 1,4-dimercaptobutane-2,3-diol.		1.95101,4-dimercaptobutane-2,3-diol;
butanediols;
dithiol;
glycol;
thiol		chelator;
human metabolite;
reducing agent
n-methylaspartate
N-Methylaspartate: An amino acid that, as the D-isomer, is the defining agonist for the NMDA receptor subtype of glutamate receptors (RECEPTORS, NMDA).. N-methyl-D-aspartic acid : An aspartic acid derivative having an N-methyl substituent and D-configuration.		2.9240amino dicarboxylic acid;
D-alpha-amino acid;
D-aspartic acid derivative;
secondary amino compound		neurotransmitter agent
manganese
Manganese: A trace element with atomic symbol Mn, atomic number 25, and atomic weight 54.94. It is concentrated in cell mitochondria, mostly in the pituitary gland, liver, pancreas, kidney, and bone, influences the synthesis of mucopolysaccharides, stimulates hepatic synthesis of cholesterol and fatty acids, and is a cofactor in many enzymes, including arginase and alkaline phosphatase in the liver. (From AMA Drug Evaluations Annual 1992, p2035). manganese(4+) : A manganese cation that is monoatomic and has a formal charge of +4.		1.9610elemental manganese;
manganese group element atom		Escherichia coli metabolite;
micronutrient
mercury
Mercury: A silver metallic element that exists as a liquid at room temperature. It has the atomic symbol Hg (from hydrargyrum, liquid silver), atomic number 80, and atomic weight 200.59. Mercury is used in many industrial applications and its salts have been employed therapeutically as purgatives, antisyphilitics, disinfectants, and astringents. It can be absorbed through the skin and mucous membranes which leads to MERCURY POISONING. Because of its toxicity, the clinical use of mercury and mercurials is diminishing.. mercury(0) : Elemental mercury of oxidation state zero.		7.7630elemental mercury;
zinc group element atom		neurotoxin
palladium
Palladium: A chemical element having an atomic weight of 106.4, atomic number of 46, and the symbol Pd. It is a white, ductile metal resembling platinum, and following it in abundance and importance of applications. It is used in dentistry in the form of gold, silver, and copper alloys.. palladium : Chemical element (nickel group element atom) with atomic number 46.		2.4620metal allergen;
nickel group element atom;
platinum group metal atom
titanium
Titanium: A dark-gray, metallic element of widespread distribution but occurring in small amounts with atomic number, 22, atomic weight, 47.867 and symbol, Ti; specific gravity, 4.5; used for fixation of fractures.		2.4110titanium group element atom
gold
Gold: A yellow metallic element with the atomic symbol Au, atomic number 79, and atomic weight 197. It is used in jewelry, goldplating of other metals, as currency, and in dental restoration. Many of its clinical applications, such as ANTIRHEUMATIC AGENTS, are in the form of its salts.		2.5120copper group element atom;
elemental gold
acetylglucosamine
Acetylglucosamine: The N-acetyl derivative of glucosamine.. N-acetyl-beta-D-glucosamine : An N-acetyl-D-glucosamine having beta-configuration at the anomeric centre.		2.0110N-acetyl-D-glucosamineepitope
deuterium
Deuterium: The stable isotope of hydrogen. It has one neutron and one proton in the nucleus.		3.69100dihydrogen
ammonium chloride
Ammonium Chloride: An acidifying agent that has expectorant and diuretic effects. Also used in etching and batteries and as a flux in electroplating.. ammonium chloride : An inorganic chloride having ammonium as the counterion.		2.0110ammonium salt;
inorganic chloride		ferroptosis inhibitor
fluorides
[no description available]		1.9510halide anion;
monoatomic fluorine
phenyl acetate
phenyl acetate: The ester formed between phenol and acetic acid. Don't confuse with phenylacetic acid derivatives listed under PHENYLACETATES.. phenyl acetate : An acetate ester obtained by the formal condensation of phenol with acetic acid.		3.67100benzenes;
phenyl acetates
cetylpyridinium chloride anhydrous
tserigel: according to first source contains polyvinylbutyral & cetylpyridinium chloride; UD only lists cetylpyridinium chloride as constituent. cetylpyridinium chloride : A pyridinium salt that has N-hexadecylpyridinium as the cation and chloride as the anion. It has antiseptic properties and is used in solutions or lozenges for the treatment of minor infections of the mouth and throat.		2.4920chloride salt;
organic chloride salt		antiseptic drug;
surfactant
transferrin
Transferrin: An iron-binding beta1-globulin that is synthesized in the LIVER and secreted into the blood. It plays a central role in the transport of IRON throughout the circulation. A variety of transferrin isoforms exist in humans, including some that are considered markers for specific disease states.		2.0210
glutamic acid
Glutamic Acid: A non-essential amino acid naturally occurring in the L-form. Glutamic acid is the most common excitatory neurotransmitter in the CENTRAL NERVOUS SYSTEM.. glutamic acid : An alpha-amino acid that is glutaric acid bearing a single amino substituent at position 2.		3.89120glutamic acid;
glutamine family amino acid;
L-alpha-amino acid;
proteinogenic amino acid		Escherichia coli metabolite;
ferroptosis inducer;
micronutrient;
mouse metabolite;
neurotransmitter;
nutraceutical
s-adenosylmethionine
acylcarnitine: structure in first source. S-adenosyl-L-methioninate : A sulfonium betaine that is a conjugate base of S-adenosyl-L-methionine obtained by the deprotonation of the carboxy group.		4.06140sulfonium betainehuman metabolite
paclitaxel
Taxus: Genus of coniferous yew trees or shrubs, several species of which have medicinal uses. Notable is the Pacific yew, Taxus brevifolia, which is used to make the anti-neoplastic drug taxol (PACLITAXEL).		3.4211taxane diterpenoid;
tetracyclic diterpenoid		antineoplastic agent;
human metabolite;
metabolite;
microtubule-stabilising agent
ng-nitroarginine methyl ester
NG-Nitroarginine Methyl Ester: A non-selective inhibitor of nitric oxide synthase. It has been used experimentally to induce hypertension.		2.7230alpha-amino acid ester;
L-arginine derivative;
methyl ester;
N-nitro compound		EC 1.14.13.39 (nitric oxide synthase) inhibitor
1-carboxyglutamic acid
1-Carboxyglutamic Acid: Found in various tissues, particularly in four blood-clotting proteins including prothrombin, in kidney protein, in bone protein, and in the protein present in various ectopic calcifications.		2.0510
captopril
Captopril: A potent and specific inhibitor of PEPTIDYL-DIPEPTIDASE A. It blocks the conversion of ANGIOTENSIN I to ANGIOTENSIN II, a vasoconstrictor and important regulator of arterial blood pressure. Captopril acts to suppress the RENIN-ANGIOTENSIN SYSTEM and inhibits pressure responses to exogenous angiotensin.. captopril : A L-proline derivative in which L-proline is substituted on nitrogen with a (2S)-2-methyl-3-sulfanylpropanoyl group. It is used as an anti-hypertensive ACE inhibitor drug.		2.0510alkanethiol;
L-proline derivative;
N-acylpyrrolidine;
pyrrolidinemonocarboxylic acid		antihypertensive agent;
EC 3.4.15.1 (peptidyl-dipeptidase A) inhibitor
fomesafen
fomesafen: a protoporphyrinogen oxidase-inhibiting herbicide. fomesafen : An N-sulfonylcarboxamide that is N-(methylsulfonyl)benzamide in which the phenyl ring is substituted by a nitro group at position 2 and a 2-chloro-4-(trifluoromethyl)phenoxy group at position 5. A protoporphyrinogen oxidase inhibitor, it was specially developed for use (generally as the corresponding sodium salt, fomesafen-sodium) for post-emergence control of broad-leaf weeds in soya.		1.9810aromatic ether;
C-nitro compound;
monochlorobenzenes;
N-sulfonylcarboxamide;
organofluorine compound;
phenols		agrochemical;
EC 1.3.3.4 (protoporphyrinogen oxidase) inhibitor;
herbicide
bidisomide
bidisomide: RN given refers to parent cpd		1.9810acetamides
3-aminoisobutyric acid
3-aminoisobutyric acid: RN given refers to cpd without isomeric designation. 3-aminoisobutyric acid : A beta-amino-acid that is isobutyric acid in which one of the methyl hydrogens is substituted by an amino group.		210amino acid zwitterion;
beta-amino acid		metabolite
thiazolyl blue
thiazolyl blue: RN & II refers to bromide. 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide : The bromide salt of 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium.		2.0210organic bromide saltcolorimetric reagent;
dye
n,n-bis(trimethylsilyl)-2,2,2-trifluoroacetamide
[no description available]		210
n-acetylvaline
N-acetylvaline: RN given refers to the (DL)-isomer		2.0110L-valine derivative;
N-acetyl-L-amino acid
triazoles
Triazoles: Heterocyclic compounds containing a five-membered ring with two carbon atoms and three nitrogen atoms with the molecular formula C2H3N3.. triazoles : An azole in which the five-membered heterocyclic aromatic skeleton contains three N atoms and two C atoms.		2.06101,2,3-triazole
phenylacetylglycine
phenylacetylglycine : A N-acylglycine that is glycine substituted on nitrogen with a phenylacetyl group.		2.0610monocarboxylic acid amide;
monocarboxylic acid;
N-acylglycine		human metabolite;
mouse metabolite
hydracrylic acid
3-hydroxypropionic acid : A 3-hydroxy monocarboxylic acid that is propionic acid in which one of the hydrogens attached to the terminal carbon is replaced by a hydroxy group.		3.39703-hydroxy monocarboxylic acid;
omega-hydroxy-short-chain fatty acid		Escherichia coli metabolite;
human metabolite
fluorodeoxyglucose f18
Fluorodeoxyglucose F18: The compound is given by intravenous injection to do POSITRON-EMISSION TOMOGRAPHY for the assessment of cerebral and myocardial glucose metabolism in various physiological or pathological states including stroke and myocardial ischemia. It is also employed for the detection of malignant tumors including those of the brain, liver, and thyroid gland. (From Martindale, The Extra Pharmacopoeia, 30th ed, p1162)		2.15102-deoxy-2-((18)F)fluoro-D-glucose;
2-deoxy-2-fluoro-aldehydo-D-glucose
beta-hydroxyisovaleric acid
3-hydroxyisovaleric acid : A 3-hydroxy monocarboxylic acid that is isovaleric acid substituted at position 3 by a hydroxy group. Used as indicator of biotin deficiency.		2.68303-hydroxy monocarboxylic acidhuman metabolite
n-acetylglutamic acid
N-acetylglutamic acid: RN given refers to (L)-isomer. N-acetyl-L-glutamic acid : An N-acyl-L-amino acid that is L-glutamic acid in which one of the amine hydrogens is substituted by an acetyl group.		1.9510N-acetyl-L-amino acid;
N-acyl-L-glutamic acid		human metabolite;
Saccharomyces cerevisiae metabolite
pentafluorobenzyl bromide
pentafluorobenzyl bromide : A member of the class benzyl bromides that is benzyl bromide in which the hydrogens at positions 2, 3, 4, 5 and 6 of the phenyl ring are replaced by fluoro groups. It is a versatile derivatization agent in chromatography and mass spectrometry.		2.0410benzyl bromides;
fluorobenzenes
propylsulfonic acid
propylsulfonic acid: RN given refers to cpd without locant for propyl moiety		2.0410
coenzyme a
[no description available]		5.52130adenosine 3',5'-bisphosphatecoenzyme;
Escherichia coli metabolite;
mouse metabolite
homocysteine
Homocysteine: A thiol-containing amino acid formed by a demethylation of METHIONINE.. homocysteine : A sulfur-containing amino acid consisting of a glycine core with a 2-mercaptoethyl side-chain.. L-homocysteine : A homocysteine that has L configuration.		21.6445680amino acid zwitterion;
homocysteine;
serine family amino acid		fundamental metabolite;
mouse metabolite
succinyl-coenzyme a
[no description available]		2.4120omega-carboxyacyl-CoAEscherichia coli metabolite;
inhibitor;
mouse metabolite
propionyl-coenzyme a
propionyl-coenzyme A: RN given refers to parent cpd		3.2360acyl-CoAEscherichia coli metabolite;
metabolite;
mouse metabolite
alpha-hydroxyisocaproic acid
alpha-hydroxyisocaproic acid: alpha-hydroxy analog of leucine; RN given refers to cpd without isomeric designation. 2-hydroxy-4-methylvaleric acid : A valeric acid derivative having a hydroxy substituent at the 2-position and a methyl substituent at the 4-position; an alpha-hydroxy analogue of leucine. A bacterial metabolite, it has also been isolated from amniotic fluid, was found in a patient with dihydrolipoyl dehydrogenase deficiency and is present in the urine of patients with short bowel syndrome.		1.96102-hydroxy fatty acid;
branched-chain fatty acid		metabolite
2-hydroxyisovaleric acid
2-hydroxyisovaleric acid: RN given refers to parent cpd without isomeric designation. 2-hydroxy-3-methylbutyric acid : A valine derivative that is valine in which the amino group has been replaced by a hydroxy group.		1.96102-hydroxy monocarboxylic acidhuman metabolite
cobalt
Cobalt: A trace element that is a component of vitamin B12. It has the atomic symbol Co, atomic number 27, and atomic weight 58.93. It is used in nuclear weapons, alloys, and pigments. Deficiency in animals leads to anemia; its excess in humans can lead to erythrocytosis.. cobalt(1+) : A monovalent inorganic cation obtained from cobalt.. cobalt atom : A cobalt group element atom that has atomic number 27.		5.66191cobalt group element atom;
metal allergen		micronutrient
vitamin b 6
Vitamin B 6: VITAMIN B 6 refers to several PICOLINES (especially PYRIDOXINE; PYRIDOXAL; & PYRIDOXAMINE) that are efficiently converted by the body to PYRIDOXAL PHOSPHATE which is a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, and aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into PYRIDOXAMINE phosphate. Although pyridoxine and Vitamin B 6 are still frequently used as synonyms, especially by medical researchers, this practice is erroneous and sometimes misleading (EE Snell; Ann NY Acad Sci, vol 585 pg 1, 1990). Most of vitamin B6 is eventually degraded to PYRIDOXIC ACID and excreted in the urine.		6.95204
diacetyldichlorofluorescein
diacetyldichlorofluorescein: stable storage form of dichlorofluorescein		2.0610
propionylcarnitine
propionylcarnitine: RN given refers to cpd without isomeric designation		7.4351O-acylcarnitineanalgesic;
antirheumatic drug;
cardiotonic drug;
human metabolite;
peripheral nervous system drug
alpha-methylphenylalanine
alpha-methylphenylalanine: induces chronic hyperphenylalaninemia in suckling rats; models for the study of inborn errors of metabolism; RN given refers to parent cpd without isomeric designation		1.9810benzenes;
monocarboxylic acid
valerates
Valerates: Derivatives of valeric acid, including its salts and esters.		5.7110short-chain fatty acid anion;
straight-chain saturated fatty acid anion		plant metabolite
1-(carboxymethylthio)tetradecane
1-(carboxymethylthio)tetradecane: structure given in first source; alkylthio acetic acid, non-beta-oxidizable		2.1310straight-chain fatty acid
caprylates
Caprylates: Derivatives of caprylic acid. Included under this heading are a broad variety of acid forms, salts, esters, and amides that contain a carboxy terminated eight carbon aliphatic structure.. octanoate : A straight-chain saturated fatty acid anion that is the conjugate base of octanoic acid (caprylic acid); believed to block adipogenesis.		210fatty acid anion 8:0;
straight-chain saturated fatty acid anion		human metabolite;
Saccharomyces cerevisiae metabolite
pentosidine
pentosidine: structure given in first source. pentosidine : An imidazopyridine having norleucine and ornithine residues attached via their side-chains at the 4- and 2-positions respectively.		2.0110imidazopyridine;
non-proteinogenic L-alpha-amino acid		biomarker;
cross-linking reagent
carglumic acid
carglumic acid: carglumic acid could be helpful in lowering plasma ammonia levels over 400 micromol/L more rapidly. carglumic acid : A urea that is the N-carbamoyl derivative of L-glutamic acid. An orphan drug used to treat a deficiency in the enzyme N-acetylglutamate synthase, which leads to acute hyperammonaemia.		10.2841N-acyl-L-glutamic acid;
ureas		carbamylphosphate synthetase I activator;
orphan drug
6-deoxyerythronolide b
6-deoxyerythronolide B: Streptomyces erythreus metabolite; presumed intermediate in biosynthesis of erythromycins; structure given in first source		2.0610erythronolide
4'-bromophenacyl triflate
[no description available]		1.9810
tiglic acid
tiglic acid : A 2-methylbut-2-enoic acid having its double bond in trans-configuration.. 2-methylbut-2-enoic acid : A branched-chain fatty acid consisting of 2-butenoic acid having a methyl group at position 2.		2.02102-methylbut-2-enoic acidplant metabolite
methotrexate
[no description available]		1.9910dicarboxylic acid;
monocarboxylic acid amide;
pteridines		abortifacient;
antimetabolite;
antineoplastic agent;
antirheumatic drug;
dermatologic drug;
DNA synthesis inhibitor;
EC 1.5.1.3 (dihydrofolate reductase) inhibitor;
immunosuppressive agent
rufinamide
rufinamide: for treatment of Lennox-Gastaut syndrome; structure in first source		7.0610aromatic amide;
heteroarene
proline
Proline: A non-essential amino acid that is synthesized from GLUTAMIC ACID. It is an essential component of COLLAGEN and is important for proper functioning of joints and tendons.. proline : An alpha-amino acid that is pyrrolidine bearing a carboxy substituent at position 2.		2.8840amino acid zwitterion;
glutamine family amino acid;
L-alpha-amino acid;
proline;
proteinogenic amino acid		algal metabolite;
compatible osmolytes;
Escherichia coli metabolite;
micronutrient;
mouse metabolite;
nutraceutical;
Saccharomyces cerevisiae metabolite
10-propargyl-10-deazaaminopterin
10-propargyl-10-deazaaminopterin: structure in first source. pralatrexate : A pteridine that is the N-4-[1-(2,4-diaminopteridin-6-yl)pent-4-yn-2-yl]benzoyl derivative of L-glutamic acid. Used for treatment of Peripheral T-Cell Lymphoma, an aggressive form of non-Hodgkins lymphoma.		3.8321N-acyl-L-glutamic acid;
pteridines;
terminal acetylenic compound		antimetabolite;
antineoplastic agent;
EC 1.5.1.3 (dihydrofolate reductase) inhibitor
docetaxel anhydrous
Docetaxel: A semisynthetic analog of PACLITAXEL used in the treatment of locally advanced or metastatic BREAST NEOPLASMS and NON-SMALL CELL LUNG CANCER.. docetaxel anhydrous : A tetracyclic diterpenoid that is paclitaxel with the N-benzyloxycarbonyl group replaced by N-tert-butoxycarbonyl, and the acetoxy group at position 10 replaced by a hydroxy group.		3.4211secondary alpha-hydroxy ketone;
tetracyclic diterpenoid		antimalarial;
antineoplastic agent;
photosensitizing agent
carbodiimides
Carbodiimides: Compounds with the general formula RN=C=NR, where R is a hydrocarbyl group.. methanediimine : A carbodiimide in which both nitrogens are unsubstituted.		1.9610carbodiimide
alpha-methyl-beta-hydroxybutyrate
2-methyl-3-hydroxybutyric acid: metabolite from isoleucine; RN given refers to cpd without isomeric designation. 3-hydroxy-2-methylbutanoic acid : A 3-hydroxy monocarboxylic acid that is butyric acid which is substituted by a methyl group and a hydroxy group at positions 2 and 3, respectively.		1.96103-hydroxy monocarboxylic acid
chlorocarbonic acid
[no description available]		2.0410organooxygen compound
2-hydroxy-3-methylvaleric acid
2-hydroxy-3-methylvaleric acid: RN given refers to cpd without isomeric designation. 2-hydroxy-3-methylpentanoic acid : A branched-chain fatty acid that is 3-methylpentanoic acid carrying a hydroxy substitutent at position 2.		1.96102-hydroxy fatty acid;
branched-chain fatty acid;
short-chain fatty acid
aminopterin
Aminopterin: A folic acid derivative used as a rodenticide that has been shown to be teratogenic.		3.8321dicarboxylic acidEC 1.5.1.3 (dihydrofolate reductase) inhibitor;
mutagen
beta-methylcrotonylglycine
beta-methylcrotonylglycine: structure. 3-methylcrotonyl glycine : An N-acylglycine in which the acyl group is specified as 3-methylbut-2-enoyl.		3.1110N-acylglycinemetabolite
biotin
vitamin B7 : Any member of a group of vitamers that belong to the chemical structural class called biotins that exhibit biological activity against vitamin B7 deficiency. Vitamin B7 deficiency is very rare in individuals who take a normal balanced diet. Foods rich in biotin are egg yolk, liver, cereals, vegetables (spinach, mushrooms) and rice. Symptoms associated with vitamin B7 deficiency include thinning hair, scaly skin rashes around eyes, nose and mouth, and brittle nails. The vitamers include biotin and its ionized and salt forms.		7.46170biotins;
vitamin B7		coenzyme;
cofactor;
Escherichia coli metabolite;
fundamental metabolite;
human metabolite;
mouse metabolite;
nutraceutical;
prosthetic group;
Saccharomyces cerevisiae metabolite
homocysteic acid
homocysteic acid: promotes growth in hypophysectomized rats; RN given refers to parent cpd. homocysteic acid : A non-proteinogenic alpha-amino acid that is homocysteine in which the thiol group has benn oxidised to the corresponding sulfonic acid.. L-homocysteic acid : A homocysteic acid with L-configuration.		7.0710homocysteic acidNMDA receptor agonist
3-hydroxyglutaric acid
3-hydroxyglutaric acid : A 3 hydroxy carboxylic acid that is glutaric acid which is substituted by a hydroxy group at position 3. It is a diagnostic marker for glutaric aciduria type I.		2.52203-hydroxy carboxylic acid;
alpha,omega-dicarboxylic acid		human blood serum metabolite;
human urinary metabolite
2-ethylhydracrylic acid
2-ethylhydracrylic acid: newly described urinary organic acid; a metabolite of L-isoleucine; structure. 2-ethylhydracrylic acid : A branched-chain saturated fatty acid that is butanoic acid substituted by a hydroxymethyl group at position 2. It is a metabolite derived from the isoleucine metabolism.		1.9610branched-chain saturated fatty acid;
hydroxy fatty acid;
short-chain fatty acid		human metabolite
cortisone
[no description available]		2.021011-oxo steroid;
17alpha-hydroxy steroid;
20-oxo steroid;
21-hydroxy steroid;
3-oxo-Delta(4) steroid;
C21-steroid;
glucocorticoid;
primary alpha-hydroxy ketone;
tertiary alpha-hydroxy ketone		human metabolite;
mouse metabolite
nsc13345
NSC13345: structure in first source		2.1310amidobenzoic acid
3-nitrotyrosine
3-nitrotyrosine: RN given refers to parent cpd without isomeric designation. 3-nitrotyrosine : A nitrotyrosine comprising tyrosine having a nitro group at the 3-position on the phenyl ring.		2.47202-nitrophenols;
C-nitro compound;
nitrotyrosine;
non-proteinogenic alpha-amino acid
s-adenosylhomocysteine
S-Adenosylhomocysteine: 5'-S-(3-Amino-3-carboxypropyl)-5'-thioadenosine. Formed from S-adenosylmethionine after transmethylation reactions.. S-adenosyl-L-homocysteine : An organic sulfide that is the S-adenosyl derivative of L-homocysteine.		8.1450adenosines;
amino acid zwitterion;
homocysteine derivative;
homocysteines;
organic sulfide		cofactor;
EC 2.1.1.72 [site-specific DNA-methyltransferase (adenine-specific)] inhibitor;
EC 2.1.1.79 (cyclopropane-fatty-acyl-phospholipid synthase) inhibitor;
epitope;
fundamental metabolite
elastin
[no description available]		2.1310oligopeptide
mevalonic acid
Mevalonic Acid: A dihydroxy monocarboxylic acid and precursor in the biosynthetic pathway known as the mevalonate pathway, which produces terpenes and steroids that are vital for diverse cellular functions.. mevalonic acid : A racemate composed of equimolar amounts of (R)- and (S)-mevalonic acid.. (R)-mevalonic acid : The (R)-enantiomer of mevalonic acid.		1.95103,5-dihydroxy-3-methylpentanoic acid
formiminoglutamic acid
Formiminoglutamic Acid: Measurement of this acid in the urine after oral administration of histidine provides the basis for the diagnostic test of folic acid deficiency and of megaloblastic anemia of pregnancy.. N-formimidoyl-L-glutamic acid : The N-formimidoyl derivative of L-glutamic acid		8.0650dicarboxylic acid;
L-glutamic acid derivative
ouabain
Ouabain: A cardioactive glycoside consisting of rhamnose and ouabagenin, obtained from the seeds of Strophanthus gratus and other plants of the Apocynaceae; used like DIGITALIS. It is commonly used in cell biological studies as an inhibitor of the NA(+)-K(+)-EXCHANGING ATPASE.. cardiac glycoside : Steroid lactones containing sugar residues that act on the contractile force of the cardiac muscles.. ouabain : A steroid hormone that is a multi-hydroxylated alpha-L-rhamnosyl cardenoloide. It binds to and inhibits the plasma membrane Na(+)/K(+)-ATPase (sodium pump). It has been isolated naturally from Strophanthus gratus.		2.081011alpha-hydroxy steroid;
14beta-hydroxy steroid;
5beta-hydroxy steroid;
alpha-L-rhamnoside;
cardenolide glycoside;
steroid hormone		anti-arrhythmia drug;
cardiotonic drug;
EC 2.3.3.1 [citrate (Si)-synthase] inhibitor;
EC 3.1.3.41 (4-nitrophenylphosphatase) inhibitor;
EC 3.6.3.10 (H(+)/K(+)-exchanging ATPase) inhibitor;
EC 3.6.3.9 (Na(+)/K(+)-transporting ATPase) inhibitor;
ion transport inhibitor;
plant metabolite
maleic acid
maleic acid: RN given refers to parent cpd(Z)-isomer which is maleic acid; all RR's given refer to (Z)-isomer; (E)-isomer is fumaric acid. maleic acid : A butenedioic acid in which the double bond has cis- (Z)-configuration.		8.6830butenedioic acidalgal metabolite;
mouse metabolite;
plant metabolite
acetyl coenzyme a
Acetyl Coenzyme A: Acetyl CoA participates in the biosynthesis of fatty acids and sterols, in the oxidation of fatty acids and in the metabolism of many amino acids. It also acts as a biological acetylating agent.		1.9510acyl-CoAacyl donor;
coenzyme;
effector;
fundamental metabolite
e-z cinnamic acid
cinnamic acid : A monocarboxylic acid that consists of acrylic acid bearing a phenyl substituent at the 3-position. It is found in Cinnamomum cassia.. trans-cinnamic acid : The E (trans) isomer of cinnamic acid		210cinnamic acidplant metabolite
arachidonic acid
icosa-5,8,11,14-tetraenoic acid : Any icosatetraenoic acid with the double bonds at positions 5, 8, 11 and 14.. arachidonate : A long-chain fatty acid anion resulting from the removal of a proton from the carboxy group of arachidonic acid.		3.821icosa-5,8,11,14-tetraenoic acid;
long-chain fatty acid;
omega-6 fatty acid		Daphnia galeata metabolite;
EC 3.1.1.1 (carboxylesterase) inhibitor;
human metabolite;
mouse metabolite
fumaric acid
fumaric acid: see also record for ferrous fumarate; use FUMARATES for general fumaric acid esters. fumaric acid : A butenedioic acid in which the C=C double bond has E geometry. It is an intermediate metabolite in the citric acid cycle.		2.6930butenedioic acidfood acidity regulator;
fundamental metabolite;
geroprotector
resveratrol
trans-resveratrol : A resveratrol in which the double bond has E configuration.		7.4110resveratrolantioxidant;
phytoalexin;
plant metabolite;
quorum sensing inhibitor;
radical scavenger
palmitoleic acid
hexadecenoate : A long-chain unsaturated fatty acid anion that is the conjugate base of hexadecenoic acid, obtained by deprotonation of the carboxy group; major species at pH 7.3.		1.9910hexadec-9-enoic acidalgal metabolite;
Daphnia galeata metabolite;
EC 3.1.1.1 (carboxylesterase) inhibitor;
Escherichia coli metabolite;
human blood serum metabolite
oleic acid
Oleic Acid: An unsaturated fatty acid that is the most widely distributed and abundant fatty acid in nature. It is used commercially in the preparation of oleates and lotions, and as a pharmaceutical solvent. (Stedman, 26th ed). oleic acid : An octadec-9-enoic acid in which the double bond at C-9 has Z (cis) stereochemistry.		3.4111octadec-9-enoic acidantioxidant;
Daphnia galeata metabolite;
EC 3.1.1.1 (carboxylesterase) inhibitor;
Escherichia coli metabolite;
mouse metabolite;
plant metabolite;
solvent
tacrolimus
Tacrolimus: A macrolide isolated from the culture broth of a strain of Streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.. tacrolimus (anhydrous) : A macrolide lactam containing a 23-membered lactone ring, originally isolated from the fermentation broth of a Japanese soil sample that contained the bacteria Streptomyces tsukubaensis.		2.4120macrolide lactambacterial metabolite;
immunosuppressive agent
ferulic acid
ferulate : A monocarboxylic acid anion obtained by the deprotonation of the carboxy group of ferulic acid.		210ferulic acidsanti-inflammatory agent;
antioxidant;
apoptosis inhibitor;
cardioprotective agent;
MALDI matrix material;
plant metabolite
pectins
Pectins: High molecular weight polysaccharides present in the cell walls of all plants. Pectins cement cell walls together. They are used as emulsifiers and stabilizers in the food industry. They have been tried for a variety of therapeutic uses including as antidiarrheals, where they are now generally considered ineffective, and in the treatment of hypercholesterolemia.. alpha-D-galacturonic acid : The alpha-anomer of D-galacturonic acid.		3.2260D-galactopyranuronic acid
riboflavin
vitamin B2 : Any member of a group of vitamers that belong to the chemical structural class called flavins that exhibit biological activity against vitamin B2 deficiency. Symptoms associated with vitamin B2 deficiency include glossitis, seborrhea, angular stomaitis, cheilosis and photophobia. The vitamers include riboflavin and its phosphate derivatives (and includes their salt, ionised and hydrate forms).		7.8572flavin;
vitamin B2		anti-inflammatory agent;
antioxidant;
cofactor;
Escherichia coli metabolite;
food colouring;
fundamental metabolite;
human urinary metabolite;
mouse metabolite;
photosensitizing agent;
plant metabolite
ammonium acetate
ammonium acetate : An ammonium salt obtained by reaction of ammonia with acetic acid. A deliquescent white crystalline solid, it has a relatively low melting point (114degreeC) for a salt. Used as a food acidity regulator, although no longer approved for this purpose in the EU.		2.0110acetate salt;
ammonium salt		buffer;
food acidity regulator
n-isovalerylglycine
N-isovalerylglycine: urinary metabolite of isovaleric acid in isovaleric acidemia (sweaty feet syndrome). N-isovalerylglycine : An N-acylglycine in which the acyl group is specified as isovaleryl.		2.0510N-acylglycinehuman urinary metabolite
isomethyleugenol
Methylation: Addition of methyl groups. In histo-chemistry methylation is used to esterify carboxyl groups and remove sulfate groups by treating tissue sections with hot methanol in the presence of hydrochloric acid. (From Stedman, 25th ed)		5.8190isomethyleugenol
malonyl coenzyme a
Malonyl Coenzyme A: A coenzyme A derivative which plays a key role in the fatty acid synthesis in the cytoplasmic and microsomal systems.. omega-carboxyacyl-CoA : An acyl-CoA that results from the formal condensation of the thiol group of coenzyme A with one of the carboxy groups of any alpha,omega-dicarboxylic acid.		5.29190malonyl-CoAsEC 2.3.1.21 (carnitine O-palmitoyltransferase) inhibitor;
Escherichia coli metabolite;
metabolite;
mouse metabolite
thiouracil
Thiouracil: Occurs in seeds of Brassica and Crucifera species. Thiouracil has been used as antithyroid, coronary vasodilator, and in congestive heart failure although its use has been largely supplanted by other drugs. It is known to cause blood dyscrasias and suspected of terato- and carcinogenesis.. thiouracil : A nucleobase analogue that is uracil in which the oxo group at C-2 is replaced by a thioxo group.		2.3720nucleobase analogue;
thiocarbonyl compound		antithyroid drug;
metabolite
sodium propionate
sodium propionate: was term of propionic acid (1986-2006). sodium propionate : An organic sodium salt comprising equal numbers of sodium and propionate ions.		2.2510organic sodium saltantifungal drug;
food preservative
n-methyl-n-(tert-butyldimethylsilyl)trifluoroacetamide
N-(tert-butyldimethylsilyl)-N-methyltrifluoroacetamide : An N-silyl compound that is N-methyltrifluoroacetamide in which the amide nitrogen is replaced by a tert-butyldimethylsilyl group.		2.0210monocarboxylic acid amide;
N-silyl compound;
trifluoroacetamide		chromatographic reagent
nadp
[no description available]		2.0210
1-pyrenyldiazomethane
1-pyrenyldiazomethane: structure given in first source		7.3920
lincomycin
Lincomycin: An antibiotic produced by Streptomyces lincolnensis var. lincolnensis. It has been used in the treatment of staphylococcal, streptococcal, and Bacteroides fragilis infections.. lincomycin : A carbohydrate-containing antibiotic produced by the actinomyces Streptomyces lincolnensis.		2.3920carbohydrate-containing antibiotic;
L-proline derivative;
monocarboxylic acid amide;
pyrrolidinecarboxamide;
S-glycosyl compound		antimicrobial agent;
bacterial metabolite
ovalbumin
Ovalbumin: An albumin obtained from the white of eggs. It is a member of the serpin superfamily.		1.9510
6-cyano-7-nitroquinoxaline-2,3-dione
6-Cyano-7-nitroquinoxaline-2,3-dione: A potent excitatory amino acid antagonist with a preference for non-NMDA iontropic receptors. It is used primarily as a research tool.		2.4120quinoxaline derivative
3-nitrophenylhydrazine
3-nitrophenylhydrazine: structure in first source		2.1510
bilirubin
[no description available]		1.9710biladienes;
dicarboxylic acid		antioxidant;
human metabolite;
mouse metabolite
dinoprostone
prostaglandin E2 : Prostaglandin F2alpha in which the hydroxy group at position 9 has been oxidised to the corresponding ketone. Prostaglandin E2 is the most common and most biologically potent of mammalian prostaglandins.		2.0710prostaglandins Ehuman metabolite;
mouse metabolite;
oxytocic
linoleic acid
Linoleic Acid: A doubly unsaturated fatty acid, occurring widely in plant glycosides. It is an essential fatty acid in mammalian nutrition and is used in the biosynthesis of prostaglandins and cell membranes. (From Stedman, 26th ed). linoleic acid : An octadecadienoic acid in which the two double bonds are at positions 9 and 12 and have Z (cis) stereochemistry.		7.3820octadecadienoic acid;
omega-6 fatty acid		algal metabolite;
Daphnia galeata metabolite;
plant metabolite
vitamin d 2
Ergocalciferols: Derivatives of ERGOSTEROL formed by ULTRAVIOLET RAYS breaking of the C9-C10 bond. They differ from CHOLECALCIFEROL in having a double bond between C22 and C23 and a methyl group at C24.. vitamin D2 : A vitamin D supplement and has been isolated from alfalfa.		3.0110hydroxy seco-steroid;
seco-ergostane;
vitamin D		bone density conservation agent;
nutraceutical;
plant metabolite;
rodenticide
cholecalciferol
Cholecalciferol: Derivative of 7-dehydroxycholesterol formed by ULTRAVIOLET RAYS breaking of the C9-C10 bond. It differs from ERGOCALCIFEROL in having a single bond between C22 and C23 and lacking a methyl group at C24.. calciol : A hydroxy seco-steroid that is (5Z,7E)-9,10-secocholesta-5,7,10(19)-triene in which the pro-S hydrogen at position 3 has been replaced by a hydroxy group. It is the inactive form of vitamin D3, being hydroxylated in the liver to calcidiol (25-hydroxyvitamin D3), which is then further hydroxylated in the kidney to give calcitriol (1,25-dihydroxyvitamin D3), the active hormone.		3.0110D3 vitamins;
hydroxy seco-steroid;
seco-cholestane;
secondary alcohol;
steroid hormone		geroprotector;
human metabolite
alpha-linolenic acid
linolenic acid : A two-membered subclass of octadecatrienoic acid comprising the (9Z,12Z,15Z)- and (6Z,9Z,12Z)-isomers. Linolenic acids are nutrients essential to the formation of prostaglandins and are also used in making paints and synthetic resins.. linolenate : A polyunsaturated fatty acid anion obtained by deprotonation of the carboxy group of either alpha- or gamma-linolenic acid.		3.821linolenic acid;
omega-3 fatty acid		micronutrient;
mouse metabolite;
nutraceutical
amphotericin b
Amphotericin B: Macrolide antifungal antibiotic produced by Streptomyces nodosus obtained from soil of the Orinoco river region of Venezuela.. amphotericin B : A macrolide antibiotic used to treat potentially life-threatening fungal infections.		1.9810antibiotic antifungal drug;
macrolide antibiotic;
polyene antibiotic		antiamoebic agent;
antiprotozoal drug;
bacterial metabolite
coenzyme q10
coenzyme Q10: Ubiquinone ring with a chain of 10 isoprene units; redox equilibrium with ubiqunol serving in mitochondrial inner membrane to transfer electrons; presence during reconstitution of acetylcholine receptor into phospholipid vesicles yields vesicles active in catalyzing carbamylcholine-sensitive Na+ flux; coenzyme Q10 depletion has been noted with use of statins. coenzyme Q10 : A ubiquinone having a side chain of 10 isoprenoid units. In the naturally occurring isomer, all isoprenyl double bonds are in the E- configuration.		2.5120ubiquinonesantioxidant;
ferroptosis inhibitor;
human metabolite
vitamin k 1
Vitamin K 1: A family of phylloquinones that contains a ring of 2-methyl-1,4-naphthoquinone and an isoprenoid side chain. Members of this group of vitamin K 1 have only one double bond on the proximal isoprene unit. Rich sources of vitamin K 1 include green plants, algae, and photosynthetic bacteria. Vitamin K1 has antihemorrhagic and prothrombogenic activity.. phylloquinone : A member of the class of phylloquinones that consists of 1,4-naphthoquinone having methyl and phytyl groups at positions 2 and 3 respectively. The parent of the class of phylloquinones.		1.9710phylloquinones;
vitamin K		cofactor;
human metabolite;
plant metabolite
sirolimus
Sirolimus: A macrolide compound obtained from Streptomyces hygroscopicus that acts by selectively blocking the transcriptional activation of cytokines thereby inhibiting cytokine production. It is bioactive only when bound to IMMUNOPHILINS. Sirolimus is a potent immunosuppressant and possesses both antifungal and antineoplastic properties.. sirolimus : A macrolide lactam isolated from Streptomyces hygroscopicus consisting of a 29-membered ring containing 4 trans double bonds, three of which are conjugated. It is an antibiotic, immunosupressive and antineoplastic agent.		2.0210antibiotic antifungal drug;
cyclic acetal;
cyclic ketone;
ether;
macrolide lactam;
organic heterotricyclic compound;
secondary alcohol		antibacterial drug;
anticoronaviral agent;
antineoplastic agent;
bacterial metabolite;
geroprotector;
immunosuppressive agent;
mTOR inhibitor
fluticasone
Fluticasone: A STEROID with GLUCOCORTICOID RECEPTOR activity that is used to manage the symptoms of ASTHMA; ALLERGIC RHINITIS, and ATOPIC DERMATITIS.. fluticasone : A trifluorinated corticosteroid used in the form of its propionate ester for treatment of allergic rhinitis.		1.971011beta-hydroxy steroid;
17alpha-hydroxy steroid;
3-oxo-Delta(4) steroid;
corticosteroid;
fluorinated steroid;
thioester		anti-allergic agent;
anti-asthmatic drug
fumarates
Fumarates: Compounds based on fumaric acid.. fumarate(2-) : A C4-dicarboxylate that is the E-isomer of but-2-enedioate(2-)		2.6930butenedioate;
C4-dicarboxylate		human metabolite;
metabolite;
Saccharomyces cerevisiae metabolite
cysteine
Cysteine: A thiol-containing non-essential amino acid that is oxidized to form CYSTINE.. L-cysteinium : The L-enantiomer of cysteinium.. cysteine : A sulfur-containing amino acid that is propanoic acid with an amino group at position 2 and a sulfanyl group at position 3.		6.53134cysteiniumfundamental metabolite
phosphorus
Phosphorus: A non-metal element that has the atomic symbol P, atomic number 15, and atomic weight 31. It is an essential element that takes part in a broad variety of biochemical reactions.		2.3620monoatomic phosphorus;
nonmetal atom;
pnictogen		macronutrient
ammonium sulfate
Ammonium Sulfate: Sulfuric acid diammonium salt. It is used in CHEMICAL FRACTIONATION of proteins.. ammonium sulfate : An inorganic sulfate salt obtained by reaction of sulfuric acid with two equivalents of ammonia. A high-melting (decomposes above 280degreeC) white solid which is very soluble in water (70.6 g/100 g water at 0degreeC; 103.8 g/100 g water at 100degreeC), it is widely used as a fertilizer for alkaline soils.		1.9510ammonium salt;
inorganic sulfate salt		fertilizer
selenium
Selenium: An element with the atomic symbol Se, atomic number 34, and atomic weight 78.97. It is an essential micronutrient for mammals and other animals but is toxic in large amounts. Selenium protects intracellular structures against oxidative damage. It is an essential component of GLUTATHIONE PEROXIDASE.		2.4220chalcogen;
nonmetal atom		micronutrient
oxalates
Oxalates: Derivatives of OXALIC ACID. Included under this heading are a broad variety of acid forms, salts, esters, and amides that are derived from the ethanedioic acid structure.		2.3720
dizocilpine maleate
Dizocilpine Maleate: A potent noncompetitive antagonist of the NMDA receptor (RECEPTORS, N-METHYL-D-ASPARTATE) used mainly as a research tool. The drug has been considered for the wide variety of neurodegenerative conditions or disorders in which NMDA receptors may play an important role. Its use has been primarily limited to animal and tissue experiments because of its psychotropic effects.. dizocilpine maleate : A maleate salt obtained by reaction of dizocilpine with one equivalent of maleic acid.		2.9240maleate salt;
tetracyclic antidepressant		anaesthetic;
anticonvulsant;
neuroprotective agent;
nicotinic antagonist;
NMDA receptor antagonist
antimycin a
Antimycin A: An antibiotic substance produced by Streptomyces species. It inhibits mitochondrial respiration and may deplete cellular levels of ATP. Antimycin A1 has been used as a fungicide, insecticide, and miticide. (From Merck Index, 12th ed). antimycin A : A nine-membered bis-lactone having methyl substituents at the 2- and 6-positions, an n-hexyl substituent at the 8-position, an acyloxy substituent at the 7-position and an aroylamido substituent at the 3-position. It is produced by Streptomyces bacteria and has found commercial use as a fish poison.		2.0210amidobenzoic acid
3-methylbutyrylcarnitine
3-methylbutyrylcarnitine: affects 2-oxo acid dehydrogenase activity in intact mitochondria of rat muscle; RN given refers to (R)-isomer. O-isovalerylcarnitine : A C5-acylcarnitine having isovaleryl as the acyl substituent.		1.9610C5-acylcarnitinehuman metabolite
hexanoylcarnitine
hexanoylcarnitine: RN given refers to chloride salt; RN for parent cpd not available 11/89. O-hexanoylcarnitine : An O-acylcarnitine compound having hexanoyl as the acyl substituent.		2.4120hexanoate ester;
O-acylcarnitine		human metabolite
bongkrekic acid
Bongkrekic Acid: An antibiotic produced by Pseudomonas cocovenenans. It is an inhibitor of MITOCHONDRIAL ADP, ATP TRANSLOCASES. Specifically, it blocks adenine nucleotide efflux from mitochondria by enhancing membrane binding.. bongkrekic acid : A tricarboxylic acid that is docosa-2,4,8,10,14,18,20-heptaenedioic acid substituted at positions 2 ,5 and 17 by methyl groups, at positions 6 by a methoxy group and at position 20 by a carboxymethyl group. It is produced by the bacterium Burkholderia gladioli and implicated in outbreaks of food-borne illness involving coconut and corn-based products in Indonesia and China.		2.0210ether;
olefinic compound;
tricarboxylic acid		apoptosis inhibitor;
ATP/ADP translocase inhibitor;
bacterial metabolite;
EC 2.5.1.18 (glutathione transferase) inhibitor;
toxin
tiglylglycine
tiglylglycine: RN is from 9th CI. tiglylglycine : An N-acylglycine that is glycine with an amine hydrogen substituted by a 2-methylbut-2-enoyl (tiglyl) group.		1.9610N-acylglycinemetabolite
tiglyl-coenzyme a
2-methylbut-2-enoyl-coenzyme A : An alk-2-enoyl-CoA that results from the formal condensation of the thiol group of coenzyme A with the carboxy group of 2-methylbut-2-enoic acid.		1.96102-enoyl-CoA;
monounsaturated fatty acyl-CoA
phosphocreatine
Phosphocreatine: An endogenous substance found mainly in skeletal muscle of vertebrates. It has been tried in the treatment of cardiac disorders and has been added to cardioplegic solutions. (Reynolds JEF(Ed): Martindale: The Extra Pharmacopoeia (electronic version). Micromedex, Inc, Englewood, CO, 1996). phosphagen : Any of a group of guanidine or amidine phosphates that function as storage depots for high-energy phosphate in muscle with the purpose of regenerating ATP from ADP during muscular contraction.. N-phosphocreatine : A phosphoamino acid consisting of creatine having a phospho group attached at the primary nitrogen of the guanidino group.		2.0110phosphagen;
phosphoamino acid		human metabolite;
mouse metabolite
mocetinostat
mocetinostat: undergoing phase II clinical trials for treatment of cancer. mocetinostat : A benzamide obtained by formal condensation of the carboxy group of 4-({[4-(pyridin-3-yl)pyrimidin-2-yl]amino}methyl)benzoic acid with one of the amino groups of benzene-1,2-diamine. It is an orally active and isotype-selective HDAC inhibitor which exhibits antitumour activity (IC50 = 0.15, 0.29, 1.66 and 0.59 muM for HDAC1, HDAC2, HDAC3 and HDAC11).		2.0610aminopyrimidine;
benzamides;
pyridines;
secondary amino compound;
secondary carboxamide;
substituted aniline		antineoplastic agent;
apoptosis inducer;
autophagy inducer;
cardioprotective agent;
EC 3.5.1.98 (histone deacetylase) inhibitor;
hepatotoxic agent
g(m2) ganglioside
G(M2) Ganglioside: A glycosphingolipid that accumulates due to a deficiency of hexosaminidase A or B (BETA-N-ACETYLHEXOSAMINIDASES), or GM2 activator protein, resulting in GANGLIOSIDOSES, heredity metabolic disorders that include TAY-SACHS DISEASE and SANDHOFF DISEASE.. ganglioside GM2 (18:0) : A sialotriaosylceramide that is N-acetyl-beta-D-galactosaminyl-(1->4)-alpha-N-acetylneuraminosyl-(2->3)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-N-acylsphingosine in which the acyl group on the sphingosine nitrogen is octadecanoyl. A constituent of natural ganglioside GM2.		2.0110N-acetyl-beta-D-galactosaminyl-(1->4)-alpha-N-acetylneuraminosyl-(2->3)-beta-D-galactosyl-(1->4)-beta-D-glucosyl-N-acylsphingosine;
sialotriaosylceramide		antigen
g(m1) ganglioside
G(M1) Ganglioside: A specific monosialoganglioside that accumulates abnormally within the nervous system due to a deficiency of GM1-b-galactosidase, resulting in GM1 gangliosidosis.. ganglioside GM1 : A sialotetraosylceramide consisting of a branched pentasaccharide made up from one sialyl residue, two galactose residues, one N-acetylgalactosamine residue and a glucose residue at the reducing end attached to N-stearoylsphingosine via a beta-linkage.		2.4220alpha-N-acetylneuraminosyl-(2->3)-[beta-D-galactosyl-(1->3)-N-acetyl-beta-D-galactosaminyl-(1->4)]-beta-D-galactosyl-(1->4)-beta-D-glucosyl-(1<->1')-N-acylsphingosine;
sialotetraosylceramide
cystathionine
Cystathionine: Sulfur-containing amino acid formed as an intermediate in the conversion of METHIONINE to CYSTEINE.. cystathionine : A modified amino acid generated by enzymic means from homocysteine and serine.		9.72274cysteine derivative
losartan potassium
Erythropoietin: Glycoprotein hormone, secreted chiefly by the KIDNEY in the adult and the LIVER in the FETUS, that acts on erythroid stem cells of the BONE MARROW to stimulate proliferation and differentiation.		2.4220
octanoylcarnitine
octanoylcarnitine: RN given refers to cpd without isomeric designation. O-octanoyl-L-carnitine : The L-enantiomer of an O-octanoylcarnitine.		2.6830O-octanoylcarnitine;
saturated fatty acyl-L-carnitine		human metabolite
calcimycin
Calcimycin: An ionophorous, polyether antibiotic from Streptomyces chartreusensis. It binds and transports CALCIUM and other divalent cations across membranes and uncouples oxidative phosphorylation while inhibiting ATPase of rat liver mitochondria. The substance is used mostly as a biochemical tool to study the role of divalent cations in various biological systems.		2.0210benzoxazole
sepharose
agarose : A linear polysaccharide made up from alternating D-galactose and 3,6-anhydro-alpha-L-galactopyranose residues joined by alpha-(1->3)- and beta-(1->4)-linkages.		1.9510
2',7'-dichlorodihydrofluorescein
2',7'-dichlorodihydrofluorescein: nonfluorescent, reduced form of 2',7'-dichlorofluorescein		2.110
nad
NAD(1-) : An anionic form of nicotinamide adenine dinucleotide arising from deprotonation of the two OH groups of the diphosphate moiety.		3.0950organophosphate oxoanioncofactor;
human metabolite;
hydrogen acceptor;
Saccharomyces cerevisiae metabolite
gastrins
Gastrins: A family of gastrointestinal peptide hormones that excite the secretion of GASTRIC JUICE. They may also occur in the central nervous system where they are presumed to be neurotransmitters.		5.1280
glucagon
Glucagon: A 29-amino acid pancreatic peptide derived from proglucagon which is also the precursor of intestinal GLUCAGON-LIKE PEPTIDES. Glucagon is secreted by PANCREATIC ALPHA CELLS and plays an important role in regulation of BLOOD GLUCOSE concentration, ketone metabolism, and several other biochemical and physiological processes. (From Gilman et al., Goodman and Gilman's The Pharmacological Basis of Therapeutics, 9th ed, p1511). glucagon : A 29-amino acid peptide hormone consisting of His, Ser, Gln, Gly, Thr, Phe, Thr, Ser, Asp, Tyr, Ser, Lys, Tyr, Leu, Asp, Ser, Arg, Arg, Ala, Gln, Asp, Phe, Val, Gln, Trp, Leu, Met, Asn and Thr residues joined in sequence.		2.420peptide hormone
cellulose
DEAE-Cellulose: Cellulose derivative used in chromatography, as ion-exchange material, and for various industrial applications.		3.0650glycoside
phosphatidylcholines
Phosphatidylcholines: Derivatives of PHOSPHATIDIC ACIDS in which the phosphoric acid is bound in ester linkage to a CHOLINE moiety.		2.66301,2-diacyl-sn-glycero-3-phosphocholine
atractyloside
Atractyloside: A glycoside of a kaurene type diterpene that is found in some plants including Atractylis gummifera (ATRACTYLIS); COFFEE; XANTHIUM, and CALLILEPIS. Toxicity is due to inhibition of ADENINE NUCLEOTIDE TRANSLOCASE.		2.0310
ubiquinone
Ubiquinone: A lipid-soluble benzoquinone which is involved in ELECTRON TRANSPORT in mitochondrial preparations. The compound occurs in the majority of aerobic organisms, from bacteria to higher plants and animals.		2.5120
sodium oxybate
Sodium Oxybate: The sodium salt of 4-hydroxybutyric acid. It is used for both induction and maintenance of ANESTHESIA.		2.910
s-adenosylmethionine
(R)-S-adenosyl-L-methionine : An S-adenosyl-L-methionine that has R-configuration.. S-adenosyl-L-methionine zwitterion : A zwitterionic tautomer of S-adenosyl-L-methionine arising from shift of the proton from the carboxy group to the amino group.. (R)-S-adenosyl-L-methionine zwitterion : An S-adenosyl-L-methionine zwitterion that has R-configuration; major species at pH 7.3.. (S)-S-adenosyl-L-methionine zwitterion : An S-adenosyl-L-methionine zwitterion that has S-configuration; major species at pH 7.3.. S-adenosyl-L-methionine : A sulfonium compound that is the S-adenosyl derivative of L-methionine. It is an intermediate in the metabolic pathway of methionine.		8.1550organic cation;
sulfonium compound		coenzyme;
cofactor;
human metabolite;
micronutrient;
Mycoplasma genitalium metabolite;
nutraceutical;
Saccharomyces cerevisiae metabolite
intrinsic factor
Intrinsic Factor: A glycoprotein secreted by the cells of the GASTRIC GLANDS that is required for the absorption of VITAMIN B 12 (cyanocobalamin). Deficiency of intrinsic factor leads to VITAMIN B 12 DEFICIENCY and ANEMIA, PERNICIOUS.		5.66100
piperidines
Piperidines: A family of hexahydropyridines.		2.420
hydroxocobalamin
Hydroxocobalamin: Injectable form of VITAMIN B 12 that has been used therapeutically to treat VITAMIN B 12 DEFICIENCY.		12.59528
methylmalonyl-coenzyme a
[no description available]		4.250
heptafluorobutyl chloroformate
heptafluorobutyl chloroformate: used to derivatize amino acids		2.0410
ascorbic acid
Ascorbic Acid: A six carbon compound related to glucose. It is found naturally in citrus fruits and many vegetables. Ascorbic acid is an essential nutrient in human diets, and necessary to maintain connective tissue and bone. Its biologically active form, vitamin C, functions as a reducing agent and coenzyme in several metabolic pathways. Vitamin C is considered an antioxidant.. L-ascorbic acid : The L-enantiomer of ascorbic acid and conjugate acid of L-ascorbate.. L-ascorbate : The L-enantiomer of ascorbate and conjugate base of L-ascorbic acid, arising from selective deprotonation of the 3-hydroxy group. Required for a range of essential metabolic reactions in all animals and plants.. vitamin C : Any member of a group of vitamers that belong to the chemical structural class called butenolides that exhibit biological activity against vitamin C deficiency in animals. The vitamers include L-ascorbic acid and its salt, ionized and oxidized forms.		3.580ascorbic acid;
vitamin C		coenzyme;
cofactor;
flour treatment agent;
food antioxidant;
food colour retention agent;
geroprotector;
plant metabolite;
skin lightening agent
warfarin
Warfarin: An anticoagulant that acts by inhibiting the synthesis of vitamin K-dependent coagulation factors. Warfarin is indicated for the prophylaxis and/or treatment of venous thrombosis and its extension, pulmonary embolism, and atrial fibrillation with embolization. It is also used as an adjunct in the prophylaxis of systemic embolism after myocardial infarction. Warfarin is also used as a rodenticide.. warfarin : A racemate comprising equal amounts of (R)- and (S)-warfarin. Extensively used as both an anticoagulant drug and as a pesticide against rats and mice.. 4-hydroxy-3-(3-oxo-1-phenylbutyl)-1-benzopyran-2-one : A member of the class of coumarins that is 4-hydroxycoumarin which is substituted at position 3 by a 1-phenyl-3-oxo-1-butyl group.		2.110benzenes;
hydroxycoumarin;
methyl ketone
antimycin
[no description available]		2.0210
transforming growth factor beta
Transforming Growth Factor beta: A factor synthesized in a wide variety of tissues. It acts synergistically with TGF-alpha in inducing phenotypic transformation and can also act as a negative autocrine growth factor. TGF-beta has a potential role in embryonal development, cellular differentiation, hormone secretion, and immune function. TGF-beta is found mostly as homodimer forms of separate gene products TGF-beta1, TGF-beta2 or TGF-beta3. Heterodimers composed of TGF-beta1 and 2 (TGF-beta1.2) or of TGF-beta2 and 3 (TGF-beta2.3) have been isolated. The TGF-beta proteins are synthesized as precursor proteins.		2.7220
cobamamide
cobamamide : A member of the class of cobalamins that is vitamin B12 in which the cyano group is replaced by a 5'-deoxyadenos-5'-yl moiety. It is one of the two metabolically active form of vitamin B12.		8.98272
vitamin b 12
Vitamin B 12: A cobalt-containing coordination compound produced by intestinal micro-organisms and found also in soil and water. Higher plants do not concentrate vitamin B 12 from the soil and so are a poor source of the substance as compared with animal tissues. INTRINSIC FACTOR is important for the assimilation of vitamin B 12.		22.5169176
levoleucovorin
Levoleucovorin: A folate analog consisting of the pharmacologically active isomer of LEUCOVORIN.. (6S)-5-formyltetrahydrofolic acid : The pharmacologically active (6S)-stereoisomer of 5-formyltetrahydrofolic acid.		3.1505-formyltetrahydrofolic acidantineoplastic agent;
metabolite
guanosine triphosphate
Guanosine Triphosphate: Guanosine 5'-(tetrahydrogen triphosphate). A guanine nucleotide containing three phosphate groups esterified to the sugar moiety.		2.0510guanosine 5'-phosphate;
purine ribonucleoside 5'-triphosphate		Escherichia coli metabolite;
mouse metabolite;
uncoupling protein inhibitor
guanine
[no description available]		5.19312-aminopurines;
oxopurine;
purine nucleobase		algal metabolite;
Escherichia coli metabolite;
human metabolite;
mouse metabolite;
Saccharomyces cerevisiae metabolite
folic acid
folcysteine: used to promote fertility in chickens. vitamin B9 : Any B-vitamin that exhibits biological activity against vitamin B9 deficiency. Vitamin B9 refers to the many forms of folic acid and its derivatives, including tetrahydrofolic acid (the active form), methyltetrahydrofolate (the primary form found in blood), methenyltetrahydrofolate, folinic acid amongst others. They are present in abundance in green leafy vegetables, citrus fruits, and animal products. Lack of vitamin B9 leads to anemia, a condition in which the body cannot produce sufficient number of red blood cells. Symptoms of vitamin B9 deficiency include fatigue, muscle weakness, and pale skin.		24.524553folic acids;
N-acyl-amino acid		human metabolite;
mouse metabolite;
nutrient
dacarbazine
(E)-dacarbazine : A dacarbazine in which the N=N double bond adopts a trans-configuration.		2.110dacarbazine
guanylyl imidodiphosphate
Guanylyl Imidodiphosphate: A non-hydrolyzable analog of GTP, in which the oxygen atom bridging the beta to the gamma phosphate is replaced by a nitrogen atom. It binds tightly to G-protein in the presence of Mg2+. The nucleotide is a potent stimulator of ADENYLYL CYCLASES.. guanosine 5'-[beta,gamma-imido]triphosphate : A nucleoside triphosphate analogue that is GTP in which the oxygen atom bridging the beta- to the gamma- phosphate is replaced by a nitrogen atom A non-hydrolyzable analog of GTP, it binds tightly to G-protein in the presence of Mg(2+).		1.9910nucleoside triphosphate analogue
5,10-methenyltetrahydrofolate
(6R)-5,10-methenyltetrahydrofolic acid : The 5,10-methenyl derivative of tetrahydrofolic acid arising from enzymatic cyclisation of 5-formyltetrahydrofolic acid.		3.5311methylenetetrahydrofolic acidEscherichia coli metabolite;
mouse metabolite
pemetrexed
pemetrexed disodium : An organic sodium salt that is the disodium salt of N-{4-[2-(2-amino-4-oxo-4,7-dihydro-1H-pyrrolo[2,3-d]pyrimidin-5-yl)ethyl]benzoyl}-L-glutamic acid. Inhibits thymidylate synthase (TS), 421 dihydrofolate reductase (DHFR), and glycinamide ribonucleotide formyltransferase (GARFT).		10.9942N-acyl-L-glutamic acid;
pyrrolopyrimidine		antimetabolite;
antineoplastic agent;
EC 1.5.1.3 (dihydrofolate reductase) inhibitor;
EC 2.1.1.45 (thymidylate synthase) inhibitor;
EC 2.1.2.2 (phosphoribosylglycinamide formyltransferase) inhibitor
sildenafil citrate
Sildenafil Citrate: A PHOSPHODIESTERASE TYPE-5 INHIBITOR; VASODILATOR AGENT and UROLOGICAL AGENT that is used in the treatment of ERECTILE DYSFUNCTION and PRIMARY PULMONARY HYPERTENSION.. sildenafil citrate : The citrate salt of sildenafil.		2.2110citrate saltEC 3.1.4.35 (3',5'-cyclic-GMP phosphodiesterase) inhibitor;
vasodilator agent
5-methyltetrahydrofolate
5-methyltetrahydrofolate : A group of heterocyclic compounds based on the 5-methyl-5,6,7,8-tetrahydropteroic acid skeleton conjugated with one or more L-glutamic acid or L-glutamate units.		4.9181
eye
[no description available]		2.4120
concanavalin a
Concanavalin A: A MANNOSE/GLUCOSE binding lectin isolated from the jack bean (Canavalia ensiformis). It is a potent mitogen used to stimulate cell proliferation in lymphocytes, primarily T-lymphocyte, cultures.		210
phosphorus radioisotopes
Phosphorus Radioisotopes: Unstable isotopes of phosphorus that decay or disintegrate emitting radiation. P atoms with atomic weights 28-34 except 31 are radioactive phosphorus isotopes.		2.6930
ConditionIndicatedRelationship StrengthStudiesTrials
Blood Pressure, High
[description not available]		04.7121
Hypertension
Persistently high systemic arterial BLOOD PRESSURE. Based on multiple readings (BLOOD PRESSURE DETERMINATION), hypertension is currently defined as when SYSTOLIC PRESSURE is consistently greater than 140 mm Hg or when DIASTOLIC PRESSURE is consistently 90 mm Hg or more.		04.7121
Innate Inflammatory Response
[description not available]		04.5751
Inflammation
A pathological process characterized by injury or destruction of tissues caused by a variety of cytologic and chemical reactions. It is usually manifested by typical signs of pain, heat, redness, swelling, and loss of function.		04.5751
Acidemia Propionic
[description not available]		08.75201
Amino Acid Metabolism Disorders, Inborn
[description not available]		015.93787
Propionic Acidemia
Autosomal recessive metabolic disorder caused by mutations in PROPIONYL-COA CARBOXYLASE genes that result in dysfunction of branch chain amino acids and of the metabolism of certain fatty acids. Neonatal clinical onset is characterized by severe metabolic acidemia accompanied by hyperammonemia, HYPERGLYCEMIA, lethargy, vomiting, HYPOTONIA; and HEPATOMEGALY. Survivors of the neonatal onset propionic acidemia often show developmental retardation, and intolerance to dietary proteins. Late-onset form of the disease shows mild mental and/or developmental retardation, sometimes without metabolic acidemia.		08.75201
CBS Deficiency
[description not available]		010.191020
Age-Related Macular Degeneration
[description not available]		03.4320
Homocystinuria
Autosomal recessive inborn error of methionine metabolism usually caused by a deficiency of CYSTATHIONINE BETA-SYNTHASE and associated with elevations of homocysteine in plasma and urine. Clinical features include a tall slender habitus, SCOLIOSIS, arachnodactyly, MUSCLE WEAKNESS, genu varus, thin blond hair, malar flush, lens dislocations, an increased incidence of MENTAL RETARDATION, and a tendency to develop fibrosis of arteries, frequently complicated by CEREBROVASCULAR ACCIDENTS and MYOCARDIAL INFARCTION. (From Adams et al., Principles of Neurology, 6th ed, p979)		010.191020
Macular Degeneration
Degenerative changes in the RETINA usually of older adults which results in a loss of vision in the center of the visual field (the MACULA LUTEA) because of damage to the retina. It occurs in dry and wet forms.		03.4320
Retinal Degeneration
A retrogressive pathological change in the retina, focal or generalized, caused by genetic defects, inflammation, trauma, vascular disease, or aging. Degeneration affecting predominantly the macula lutea of the retina is MACULAR DEGENERATION. (Newell, Ophthalmology: Principles and Concepts, 7th ed, p304)		04.4380
Polyneuropathy, Acquired
[description not available]		04.3741
Deficiency, Vitamin B 12
[description not available]		022.4358286
Polyneuropathies
Diseases of multiple peripheral nerves simultaneously. Polyneuropathies usually are characterized by symmetrical, bilateral distal motor and sensory impairment with a graded increase in severity distally. The pathological processes affecting peripheral nerves include degeneration of the axon, myelin or both. The various forms of polyneuropathy are categorized by the type of nerve affected (e.g., sensory, motor, or autonomic), by the distribution of nerve injury (e.g., distal vs. proximal), by nerve component primarily affected (e.g., demyelinating vs. axonal), by etiology, or by pattern of inheritance.		04.3741
Vitamin B 12 Deficiency
A nutritional condition produced by a deficiency of VITAMIN B 12 in the diet, characterized by megaloblastic anemia. Since vitamin B 12 is not present in plants, humans have obtained their supply from animal products, from multivitamin supplements in the form of pills, and as additives to food preparations. A wide variety of neuropsychiatric abnormalities is also seen in vitamin B 12 deficiency and appears to be due to an undefined defect involving myelin synthesis. (From Cecil Textbook of Medicine, 19th ed, p848)		027.4358286
Hyperhomocysteinemia
Condition in which the plasma levels of homocysteine and related metabolites are elevated (		08.99377
Acute Kidney Failure
[description not available]		03.4270
Atypical Hemolytic-Uremic Syndrome
[description not available]		03.5520
Thrombotic Microangiopathies
Diseases that result in THROMBOSIS in MICROVASCULATURE. The two most prominent diseases are PURPURA, THROMBOTIC THROMBOCYTOPENIC; and HEMOLYTIC-UREMIC SYNDROME. Multiple etiological factors include VASCULAR ENDOTHELIAL CELL damage due to SHIGA TOXIN; FACTOR H deficiency; and aberrant VON WILLEBRAND FACTOR formation.		02.9130
Acute Kidney Injury
Abrupt reduction in kidney function. Acute kidney injury encompasses the entire spectrum of the syndrome including acute kidney failure; ACUTE KIDNEY TUBULAR NECROSIS; and other less severe conditions.		03.4270
Atypical Hemolytic Uremic Syndrome
An hereditary hemolytic uremic syndrome associated with variations in the gene that encodes COMPLEMENT FACTOR H, or the related proteins CFHR1 and CFHR3. Disease often progresses to CHRONIC KIDNEY FAILURE without the prodromal symptoms of ENTEROCOLITIS and DIARRHEA that characterize typical hemolytic uremic syndrome.		08.5520
Diabetes Mellitus, Adult-Onset
[description not available]		06.8281
Diabetes Mellitus, Type 2
A subclass of DIABETES MELLITUS that is not INSULIN-responsive or dependent (NIDDM). It is characterized initially by INSULIN RESISTANCE and HYPERINSULINEMIA; and eventually by GLUCOSE INTOLERANCE; HYPERGLYCEMIA; and overt diabetes. Type II diabetes mellitus is no longer considered a disease exclusively found in adults. Patients seldom develop KETOSIS but often exhibit OBESITY.		06.8281
Inborn Errors of Metabolism
[description not available]		015.412304
Metabolism, Inborn Errors
Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.		015.412304
Neoplastic Processes
The pathological mechanisms and forms taken by tissue during degeneration into a neoplasm and its subsequent activity.		02.6920
Aging
The gradual irreversible changes in structure and function of an organism that occur as a result of the passage of time.		010.65394
Autism Spectrum Disorder
Wide continuum of associated cognitive and neurobehavioral disorders, including, but not limited to, three core-defining features: impairments in socialization, impairments in verbal and nonverbal communication, and restricted and repetitive patterns of behaviors. (from DSM-V)		03.3310
Cardiometabolic Syndrome
A cluster of symptoms that are risk factors for CARDIOVASCULAR DISEASES and TYPE 2 DIABETES MELLITUS. The major components not only include metabolic dysfunctions of METABOLIC SYNDROME but also HYPERTENSION, and ABDOMINAL OBESITY.		03.7230
Metabolic Syndrome
A cluster of symptoms that are risk factors for CARDIOVASCULAR DISEASES and TYPE 2 DIABETES MELLITUS. The major components of metabolic syndrome include ABDOMINAL OBESITY; atherogenic DYSLIPIDEMIA; HYPERTENSION; HYPERGLYCEMIA; INSULIN RESISTANCE; a proinflammatory state; and a prothrombotic (THROMBOSIS) state.		03.7230
Decreased Muscle Tone
[description not available]		05.3980
Pregnancy
The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.		016.1513321
Benign Neoplasms
[description not available]		06.83131
Neoplasms
New abnormal growth of tissue. Malignant neoplasms show a greater degree of anaplasia and have the properties of invasion and metastasis, compared to benign neoplasms.		06.83131
Cognitive Decline
[description not available]		07.0191
Pericementitis
[description not available]		02.4110
Periodontitis
Inflammation and loss of connective tissues supporting or surrounding the teeth. This may involve any part of the PERIODONTIUM. Periodontitis is currently classified by disease progression (CHRONIC PERIODONTITIS; AGGRESSIVE PERIODONTITIS) instead of age of onset. (From 1999 International Workshop for a Classification of Periodontal Diseases and Conditions, American Academy of Periodontology)		02.4110
Cognitive Dysfunction
Diminished or impaired mental and/or intellectual function.		07.0191
Nervous System Disorders
[description not available]		09.4212
Nervous System Diseases
Diseases of the central and peripheral nervous system. This includes disorders of the brain, spinal cord, cranial nerves, peripheral nerves, nerve roots, autonomic nervous system, neuromuscular junction, and muscle.		09.4212
Chronic Kidney Failure
[description not available]		08.92353
Deficiency, Mental
[description not available]		03.690
Kidney Failure, Chronic
The end-stage of CHRONIC RENAL INSUFFICIENCY. It is characterized by the severe irreversible kidney damage (as measured by the level of PROTEINURIA) and the reduction in GLOMERULAR FILTRATION RATE to less than 15 ml per min (Kidney Foundation: Kidney Disease Outcome Quality Initiative, 2002). These patients generally require HEMODIALYSIS or KIDNEY TRANSPLANTATION.		08.92353
Intellectual Disability
Subnormal intellectual functioning which originates during the developmental period. This has multiple potential etiologies, including genetic defects and perinatal insults. Intelligence quotient (IQ) scores are commonly used to determine whether an individual has an intellectual disability. IQ scores between 70 and 79 are in the borderline range. Scores below 67 are in the disabled range. (from Joynt, Clinical Neurology, 1992, Ch55, p28)		03.690
Birth Weight
The mass or quantity of heaviness of an individual at BIRTH. It is expressed by units of pounds or kilograms.		03.0140
Glial Cell Tumors
[description not available]		02.610
Glioma
Benign and malignant central nervous system neoplasms derived from glial cells (i.e., astrocytes, oligodendrocytes, and ependymocytes). Astrocytes may give rise to astrocytomas (ASTROCYTOMA) or glioblastoma multiforme (see GLIOBLASTOMA). Oligodendrocytes give rise to oligodendrogliomas (OLIGODENDROGLIOMA) and ependymocytes may undergo transformation to become EPENDYMOMA; CHOROID PLEXUS NEOPLASMS; or colloid cysts of the third ventricle. (From Escourolle et al., Manual of Basic Neuropathology, 2nd ed, p21)		07.610
Canine Diseases
[description not available]		05.88122
Pancreatic Insufficiency
[description not available]		03.9421
Bowel Diseases, Inflammatory
[description not available]		03.811
Exocrine Pancreatic Insufficiency
A malabsorption condition resulting from greater than 10% reduction in the secretion of pancreatic digestive enzymes (LIPASE; PROTEASES; and AMYLASE) by the EXOCRINE PANCREAS into the DUODENUM. This condition is often associated with CYSTIC FIBROSIS and with chronic PANCREATITIS.		03.9421
Inflammatory Bowel Diseases
Chronic, non-specific inflammation of the GASTROINTESTINAL TRACT. Etiology may be genetic or environmental. This term includes CROHN DISEASE and ULCERATIVE COLITIS.		03.811
Drug Refractory Epilepsy
[description not available]		02.4110
Breast Cancer
[description not available]		04.1131
Breast Neoplasms
Tumors or cancer of the human BREAST.		04.1131
Menopause
The last menstrual period. Permanent cessation of menses (MENSTRUATION) is usually defined after 6 to 12 months of AMENORRHEA in a woman over 45 years of age. In the United States, menopause generally occurs in women between 48 and 55 years of age.		02.610
Electron Transport Chain Deficiencies, Mitochondrial
[description not available]		03.5980
Mitochondrial Diseases
Diseases caused by abnormal function of the MITOCHONDRIA. They may be caused by mutations, acquired or inherited, in mitochondrial DNA or in nuclear genes that code for mitochondrial components. They may also be the result of acquired mitochondria dysfunction due to adverse effects of drugs, infections, or other environmental causes.		03.5980
Neuropathy, Subacute Combined Degeneration
[description not available]		04.1650
Myelopathy
[description not available]		03.5280
Spinal Cord Diseases
Pathologic conditions which feature SPINAL CORD damage or dysfunction, including disorders involving the meninges and perimeningeal spaces surrounding the spinal cord. Traumatic injuries, vascular diseases, infections, and inflammatory/autoimmune processes may affect the spinal cord.		03.5280
Coronary Heart Disease
[description not available]		09.551
Cardiovascular Stroke
[description not available]		02.7630
Coronary Disease
An imbalance between myocardial functional requirements and the capacity of the CORONARY VESSELS to supply sufficient blood flow. It is a form of MYOCARDIAL ISCHEMIA (insufficient blood supply to the heart muscle) caused by a decreased capacity of the coronary vessels.		04.551
Myocardial Infarction
NECROSIS of the MYOCARDIUM caused by an obstruction of the blood supply to the heart (CORONARY CIRCULATION).		02.7630
Chemical Dependence
[description not available]		03.7430
Substance-Related Disorders
Disorders related to substance use or abuse.		03.7430
Chronic Kidney Diseases
[description not available]		04.4240
Renal Insufficiency, Chronic
Conditions in which the KIDNEYS perform below the normal level for more than three months. Chronic kidney insufficiency is classified by five stages according to the decline in GLOMERULAR FILTRATION RATE and the degree of kidney damage (as measured by the level of PROTEINURIA). The most severe form is the end-stage renal disease (CHRONIC KIDNEY FAILURE). (Kidney Foundation: Kidney Disease Outcome Quality Initiative, 2002)		04.4240
Cat Diseases
Diseases of the domestic cat (Felis catus or F. domesticus). This term does not include diseases of the so-called big cats such as CHEETAHS; LIONS; tigers, cougars, panthers, leopards, and other Felidae for which the heading CARNIVORA is used.		03.3160
Hyperammonemia
Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA.		03.85110
Acute Confusional Senile Dementia
[description not available]		04.9280
Alzheimer Disease
A degenerative disease of the BRAIN characterized by the insidious onset of DEMENTIA. Impairment of MEMORY, judgment, attention span, and problem solving skills are followed by severe APRAXIAS and a global loss of cognitive abilities. The condition primarily occurs after age 60, and is marked pathologically by severe cortical atrophy and the triad of SENILE PLAQUES; NEUROFIBRILLARY TANGLES; and NEUROPIL THREADS. (From Adams et al., Principles of Neurology, 6th ed, pp1049-57)		04.9280
Disease Exacerbation
[description not available]		05.51150
Orphan Diseases
Rare diseases that have not been well studied.		03.4820
Congenital Immunodeficiency Disease
[description not available]		02.610
Abscess
Accumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection.		02.610
Primary Immunodeficiency Diseases
Genetic immunologic deficiency diseases and syndromes due to mutations in genes involved in IMMUNITY generally characterized by an increased susceptibility to infectious diseases. They are often associated with AUTOIMMUNE DISEASE manifestations.		02.610
Cardiovascular Diseases
Pathological conditions involving the CARDIOVASCULAR SYSTEM including the HEART; the BLOOD VESSELS; or the PERICARDIUM.		05.7773
Abdominal Migraine
[description not available]		02.2110
Migraine Disorders
A class of disabling primary headache disorders, characterized by recurrent unilateral pulsatile headaches. The two major subtypes are common migraine (without aura) and classic migraine (with aura or neurological symptoms). (International Classification of Headache Disorders, 2nd ed. Cephalalgia 2004: suppl 1)		02.2110
Late Onset Diseases
[description not available]		02.2110
Ataxia
Impairment of the ability to perform smoothly coordinated voluntary movements. This condition may affect the limbs, trunk, eyes, pharynx, larynx, and other structures. Ataxia may result from impaired sensory or motor function. Sensory ataxia may result from posterior column injury or PERIPHERAL NERVE DISEASES. Motor ataxia may be associated with CEREBELLAR DISEASES; CEREBRAL CORTEX diseases; THALAMIC DISEASES; BASAL GANGLIA DISEASES; injury to the RED NUCLEUS; and other conditions.		02.4220
Genetic Predisposition
[description not available]		03.5880
Disease Models, Animal
Naturally-occurring or experimentally-induced animal diseases with pathological processes analogous to human diseases.		05.3200
Anxiety
Feelings or emotions of dread, apprehension, and impending disaster but not disabling as with ANXIETY DISORDERS.		02.2510
Acid beta-Glucosidase Deficiency
[description not available]		03.6530
Gaucher Disease
An autosomal recessive disorder caused by a deficiency of acid beta-glucosidase (GLUCOSYLCERAMIDASE) leading to intralysosomal accumulation of glycosylceramide mainly in cells of the MONONUCLEAR PHAGOCYTE SYSTEM. The characteristic Gaucher cells, glycosphingolipid-filled HISTIOCYTES, displace normal cells in BONE MARROW and visceral organs causing skeletal deterioration, hepatosplenomegaly, and organ dysfunction. There are several subtypes based on the presence and severity of neurological involvement.		03.6530
Addison's Anemia
[description not available]		06.55210
Deficiency, Folic Acid
[description not available]		016.637924
Folic Acid Deficiency
A nutritional condition produced by a deficiency of FOLIC ACID in the diet. Many plant and animal tissues contain folic acid, abundant in green leafy vegetables, yeast, liver, and mushrooms but destroyed by long-term cooking. Alcohol interferes with its intermediate metabolism and absorption. Folic acid deficiency may develop in long-term anticonvulsant therapy or with use of oral contraceptives. This deficiency causes anemia, macrocytic anemia, and megaloblastic anemia. It is indistinguishable from vitamin B 12 deficiency in peripheral blood and bone marrow findings, but the neurologic lesions seen in B 12 deficiency do not occur. (Merck Manual, 16th ed)		016.637924
Gastritis, Atrophic
GASTRITIS with atrophy of the GASTRIC MUCOSA, the GASTRIC PARIETAL CELLS, and the mucosal glands leading to ACHLORHYDRIA. Atrophic gastritis usually progresses from chronic gastritis.		04.64100
Malnourishment
[description not available]		05.3641
Action Tremor
[description not available]		04.7921
Tremor
Cyclical movement of a body part that can represent either a physiologic process or a manifestation of disease. Intention or action tremor, a common manifestation of CEREBELLAR DISEASES, is aggravated by movement. In contrast, resting tremor is maximal when there is no attempt at voluntary movement, and occurs as a relatively frequent manifestation of PARKINSON DISEASE.		04.7921
Malnutrition
An imbalanced nutritional status resulting from insufficient intake of nutrients to meet normal physiological requirement.		010.3641
Short Bowel Syndrome
A malabsorption syndrome resulting from extensive operative resection of the SMALL INTESTINE, the absorptive region of the GASTROINTESTINAL TRACT.		03.1550
Sensitivity and Specificity
Binary classification measures to assess test results. Sensitivity or recall rate is the proportion of true positives. Specificity is the probability of correctly determining the absence of a condition. (From Last, Dictionary of Epidemiology, 2d ed)		09.06462
Idiopathic Parkinson Disease
[description not available]		010.3170
Peripheral Nerve Diseases
[description not available]		04.77110
Parkinson Disease
A progressive, degenerative neurologic disease characterized by a TREMOR that is maximal at rest, retropulsion (i.e. a tendency to fall backwards), rigidity, stooped posture, slowness of voluntary movements, and a masklike facial expression. Pathologic features include loss of melanin containing neurons in the substantia nigra and other pigmented nuclei of the brainstem. LEWY BODIES are present in the substantia nigra and locus coeruleus but may also be found in a related condition (LEWY BODY DISEASE, DIFFUSE) characterized by dementia in combination with varying degrees of parkinsonism. (Adams et al., Principles of Neurology, 6th ed, p1059, pp1067-75)		010.3170
Peripheral Nervous System Diseases
Diseases of the peripheral nerves external to the brain and spinal cord, which includes diseases of the nerve roots, ganglia, plexi, autonomic nerves, sensory nerves, and motor nerves.		04.77110
Brain Diseases, Metabolic, Familial
[description not available]		05.0490
Pulmonary Hypertension
[description not available]		07.2510
Hypertension, Pulmonary
Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES.		02.2510
BH4 Deficiency
[description not available]		05.62130
Phenylketonurias
A group of autosomal recessive disorders marked by a deficiency of the hepatic enzyme PHENYLALANINE HYDROXYLASE or less frequently by reduced activity of DIHYDROPTERIDINE REDUCTASE (i.e., atypical phenylketonuria). Classical phenylketonuria is caused by a severe deficiency of phenylalanine hydroxylase and presents in infancy with developmental delay; SEIZURES; skin HYPOPIGMENTATION; ECZEMA; and demyelination in the central nervous system. (From Adams et al., Principles of Neurology, 6th ed, p952).		05.62130
Cardiomyopathy, Congestive
[description not available]		02.2510
Cardiomyopathy, Dilated
A form of CARDIAC MUSCLE disease that is characterized by ventricular dilation, VENTRICULAR DYSFUNCTION, and HEART FAILURE. Risk factors include SMOKING; ALCOHOL DRINKING; HYPERTENSION; INFECTION; PREGNANCY; and mutations in the LMNA gene encoding LAMIN TYPE A, a NUCLEAR LAMINA protein.		02.2510
Metastase
[description not available]		02.420
Invasiveness, Neoplasm
[description not available]		02.2510
Neoplasm Metastasis
The transfer of a neoplasm from one organ or part of the body to another remote from the primary site.		02.420
Cholera Infantum
[description not available]		03.360
Proteinuria
The presence of proteins in the urine, an indicator of KIDNEY DISEASES.		08.360
Anemia, Macrocytic
Anemia characterized by larger than normal erythrocytes, increased mean corpuscular volume (MCV) and increased mean corpuscular hemoglobin (MCH).		04.9140
Insulin Sensitivity
[description not available]		05.3340
Insulin Resistance
Diminished effectiveness of INSULIN in lowering blood sugar levels: requiring the use of 200 units or more of insulin per day to prevent HYPERGLYCEMIA or KETOSIS.		05.3340
Asymmetric Diabetic Proximal Motor Neuropathy
[description not available]		02.9340
Diabetic Neuropathies
Peripheral, autonomic, and cranial nerve disorders that are associated with DIABETES MELLITUS. These conditions usually result from diabetic microvascular injury involving small blood vessels that supply nerves (VASA NERVORUM). Relatively common conditions which may be associated with diabetic neuropathy include third nerve palsy (see OCULOMOTOR NERVE DISEASES); MONONEUROPATHY; mononeuropathy multiplex; diabetic amyotrophy; a painful POLYNEUROPATHY; autonomic neuropathy; and thoracoabdominal neuropathy. (From Adams et al., Principles of Neurology, 6th ed, p1325)		02.9340
Post-Natal Depression
[description not available]		02.3110
Depression, Postpartum
Depression in POSTPARTUM WOMEN, usually within four weeks after giving birth (PARTURITION). The degree of depression ranges from mild transient depression to neurotic or psychotic depressive disorders. (From DSM-IV, p386)		02.3110
Cardiac Failure
[description not available]		02.5520
Heart Failure
A heterogeneous condition in which the heart is unable to pump out sufficient blood to meet the metabolic need of the body. Heart failure can be caused by structural defects, functional abnormalities (VENTRICULAR DYSFUNCTION), or a sudden overload beyond its capacity. Chronic heart failure is more common than acute heart failure which results from sudden insult to cardiac function, such as MYOCARDIAL INFARCTION.		07.5520
Aqueductal Stenosis
[description not available]		02.9440
Neuroblastoma
A common neoplasm of early childhood arising from neural crest cells in the sympathetic nervous system, and characterized by diverse clinical behavior, ranging from spontaneous remission to rapid metastatic progression and death. This tumor is the most common intraabdominal malignancy of childhood, but it may also arise from thorax, neck, or rarely occur in the central nervous system. Histologic features include uniform round cells with hyperchromatic nuclei arranged in nests and separated by fibrovascular septa. Neuroblastomas may be associated with the opsoclonus-myoclonus syndrome. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, pp2099-2101; Curr Opin Oncol 1998 Jan;10(1):43-51)		07.4320
Child Mental Disorders
[description not available]		02.1510
Neurodevelopmental Disorders
These are a group of conditions with onset in the developmental period. The disorders typically manifest early in development, often before the child enters grade school, and are characterized by developmental deficits that produce impairments of personal, social, academic, or occupational functioning. (From DSM-5).		02.1510
Complications, Pregnancy
[description not available]		05.88230
Genetic Diseases, X-Chromosome Linked
[description not available]		02.1510
Glycine Encephalopathy
[description not available]		02.4520
Retinal Diseases
Diseases involving the RETINA.		02.4820
Body Weight
The mass or quantity of heaviness of an individual. It is expressed by units of pounds or kilograms.		05.61290
Anemia, Megaloblastic
A disorder characterized by the presence of ANEMIA, abnormally large red blood cells (megalocytes or macrocytes), and MEGALOBLASTS.		06.96280
Dysesthesia
[description not available]		04.260
Psychoses
[description not available]		02.8830
Depression
Depressive states usually of moderate intensity in contrast with MAJOR DEPRESSIVE DISORDER present in neurotic and psychotic disorders.		02.7830
Psychotic Disorders
Disorders in which there is a loss of ego boundaries or a gross impairment in reality testing with delusions or prominent hallucinations. (From DSM-IV, 1994)		02.8830
Nerve Pain
[description not available]		03.4620
Neuralgia
Intense or aching pain that occurs along the course or distribution of a peripheral or cranial nerve.		03.4620
Morbid Obesity
[description not available]		03.9940
Obesity, Morbid
The condition of weighing two, three, or more times the ideal weight, so called because it is associated with many serious and life-threatening disorders. In the BODY MASS INDEX, morbid obesity is defined as having a BMI greater than 40.0 kg/m2.		03.9940
Abnormalities, Congenital
[description not available]		03.0610
Colitis Gravis
[description not available]		02.5320
Colitis, Granulomatous
[description not available]		02.9140
Colitis, Ulcerative
Inflammation of the COLON that is predominantly confined to the MUCOSA. Its major symptoms include DIARRHEA, rectal BLEEDING, the passage of MUCUS, and ABDOMINAL PAIN.		02.5320
Crohn Disease
A chronic transmural inflammation that may involve any part of the DIGESTIVE TRACT from MOUTH to ANUS, mostly found in the ILEUM, the CECUM, and the COLON. In Crohn disease, the inflammation, extending through the intestinal wall from the MUCOSA to the serosa, is characteristically asymmetric and segmental. Epithelioid GRANULOMAS may be seen in some patients.		02.9140
Bacterial Overgrowth Syndrome
[description not available]		02.1710
Enterovirus Infections
Diseases caused by ENTEROVIRUS.		02.1510
Acquired Metabolic Diseases, Brain
[description not available]		04.5590
Sensation Disorders
Disorders of the special senses (i.e., VISION; HEARING; TASTE; and SMELL) or somatosensory system (i.e., afferent components of the PERIPHERAL NERVOUS SYSTEM).		02.4420
Deficiency, Vitamin D
[description not available]		04.4750
Vitamin D Deficiency
A nutritional condition produced by a deficiency of VITAMIN D in the diet, insufficient production of vitamin D in the skin, inadequate absorption of vitamin D from the diet, or abnormal conversion of vitamin D to its bioactive metabolites. It is manifested clinically as RICKETS in children and OSTEOMALACIA in adults. (From Cecil Textbook of Medicine, 19th ed, p1406)		04.4750
Brain Disorders
[description not available]		05.9291
Atrophy
Decrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes.		03.2660
Brain Diseases
Pathologic conditions affecting the BRAIN, which is composed of the intracranial components of the CENTRAL NERVOUS SYSTEM. This includes (but is not limited to) the CEREBRAL CORTEX; intracranial white matter; BASAL GANGLIA; THALAMUS; HYPOTHALAMUS; BRAIN STEM; and CEREBELLUM.		05.9291
Avitaminosis
A condition due to a deficiency of one or more essential vitamins. (Dorland, 27th ed)		05.6971
Complication, Postoperative
[description not available]		03.83110
Postoperative Complications
Pathologic processes that affect patients after a surgical procedure. They may or may not be related to the disease for which the surgery was done, and they may or may not be direct results of the surgery.		03.83110
Symptom Cluster
[description not available]		05.390
Syndrome
A characteristic symptom complex.		05.390
Anemias, Iron-Deficiency
[description not available]		03.9240
Anemia, Iron-Deficiency
Anemia characterized by decreased or absent iron stores, low serum iron concentration, low transferrin saturation, and low hemoglobin concentration or hematocrit value. The erythrocytes are hypochromic and microcytic and the iron binding capacity is increased.		03.9240
Malabsorption Syndromes
General term for a group of MALNUTRITION syndromes caused by failure of normal INTESTINAL ABSORPTION of nutrients.		06.11110
Cancer of Esophagus
[description not available]		02.1510
Esophageal Neoplasms
Tumors or cancer of the ESOPHAGUS.		02.1510
Acute Hypercapnic Respiratory Failure
[description not available]		02.1710
Respiratory Insufficiency
Failure to adequately provide oxygen to cells of the body and to remove excess carbon dioxide from them. (Stedman, 25th ed)		02.1710
Acute Brain Injuries
[description not available]		02.1710
Brain Injuries
Acute and chronic (see also BRAIN INJURIES, CHRONIC) injuries to the brain, including the cerebral hemispheres, CEREBELLUM, and BRAIN STEM. Clinical manifestations depend on the nature of injury. Diffuse trauma to the brain is frequently associated with DIFFUSE AXONAL INJURY or COMA, POST-TRAUMATIC. Localized injuries may be associated with NEUROBEHAVIORAL MANIFESTATIONS; HEMIPARESIS, or other focal neurologic deficits.		02.1710
Preterm Birth
[description not available]		02.4720
Premature Birth
CHILDBIRTH before 37 weeks of PREGNANCY (259 days from the first day of the mother's last menstrual period, or 245 days after FERTILIZATION).		02.4720
Intestinal Diseases
Pathological processes in any segment of the INTESTINE from DUODENUM to RECTUM.		03.8921
Clasp-Knife Spasticity
[description not available]		02.6120
Muscle Spasticity
A form of muscle hypertonia associated with upper MOTOR NEURON DISEASE. Resistance to passive stretch of a spastic muscle results in minimal initial resistance (a free interval) followed by an incremental increase in muscle tone. Tone increases in proportion to the velocity of stretch. Spasticity is usually accompanied by HYPERREFLEXIA and variable degrees of MUSCLE WEAKNESS. (From Adams et al., Principles of Neurology, 6th ed, p54)		02.6120
Cancer of Stomach
[description not available]		02.4920
Stomach Neoplasms
Tumors or cancer of the STOMACH.		02.4920
Besnier-Boeck Disease
[description not available]		02.2110
Thrombopenia
[description not available]		02.2110
Autoimmune Thrombocytopenia
[description not available]		02.2110
Sarcoidosis
An idiopathic systemic inflammatory granulomatous disorder comprised of epithelioid and multinucleated giant cells with little necrosis. It usually invades the lungs with fibrosis and may also involve lymph nodes, skin, liver, spleen, eyes, phalangeal bones, and parotid glands.		02.2110
Thrombocytopenia
A subnormal level of BLOOD PLATELETS.		02.2110
Purpura, Thrombocytopenic, Idiopathic
Thrombocytopenia occurring in the absence of toxic exposure or a disease associated with decreased platelets. It is mediated by immune mechanisms, in most cases IMMUNOGLOBULIN G autoantibodies which attach to platelets and subsequently undergo destruction by macrophages. The disease is seen in acute (affecting children) and chronic (adult) forms.		02.2110
Kidney Failure
A severe irreversible decline in the ability of kidneys to remove wastes, concentrate URINE, and maintain ELECTROLYTE BALANCE; BLOOD PRESSURE; and CALCIUM metabolism.		04.2260
Heritable Pulmonary Arterial Hypertension
[description not available]		02.2110
Cyanosis
A bluish or purplish discoloration of the skin and mucous membranes due to an increase in the amount of deoxygenated hemoglobin in the blood or a structural defect in the hemoglobin molecule.		02.2110
Pyrexia
[description not available]		02.4820
Tachyarrhythmia
[description not available]		02.2110
Fever
An abnormal elevation of body temperature, usually as a result of a pathologic process.		02.4820
Tachycardia
Abnormally rapid heartbeat, usually with a HEART RATE above 100 beats per minute for adults. Tachycardia accompanied by disturbance in the cardiac depolarization (cardiac arrhythmia) is called tachyarrhythmia.		02.2110
Renal Insufficiency
Conditions in which the KIDNEYS perform below the normal level in the ability to remove wastes, concentrate URINE, and maintain ELECTROLYTE BALANCE; BLOOD PRESSURE; and CALCIUM metabolism. Renal insufficiency can be classified by the degree of kidney damage (as measured by the level of PROTEINURIA) and reduction in GLOMERULAR FILTRATION RATE.		04.2260
Familial Primary Pulmonary Hypertension
Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease.		02.2110
Apoplexy
[description not available]		03.4470
Amentia
[description not available]		06.6281
Dementia
An acquired organic mental disorder with loss of intellectual abilities of sufficient severity to interfere with social or occupational functioning. The dysfunction is multifaceted and involves memory, behavior, personality, judgment, attention, spatial relations, language, abstract thought, and other executive functions. The intellectual decline is usually progressive, and initially spares the level of consciousness.		06.6281
Stroke
A group of pathological conditions characterized by sudden, non-convulsive loss of neurological function due to BRAIN ISCHEMIA or INTRACRANIAL HEMORRHAGES. Stroke is classified by the type of tissue NECROSIS, such as the anatomic location, vasculature involved, etiology, age of the affected individual, and hemorrhagic vs. non-hemorrhagic nature. (From Adams et al., Principles of Neurology, 6th ed, pp777-810)		03.4470
47,XX,+21
[description not available]		03.3260
Down Syndrome
A chromosome disorder associated either with an extra chromosome 21 or an effective trisomy for chromosome 21. Clinical manifestations include hypotonia, short stature, brachycephaly, upslanting palpebral fissures, epicanthus, Brushfield spots on the iris, protruding tongue, small ears, short, broad hands, fifth finger clinodactyly, Simian crease, and moderate to severe INTELLECTUAL DISABILITY. Cardiac and gastrointestinal malformations, a marked increase in the incidence of LEUKEMIA, and the early onset of ALZHEIMER DISEASE are also associated with this condition. Pathologic features include the development of NEUROFIBRILLARY TANGLES in neurons and the deposition of AMYLOID BETA-PROTEIN, similar to the pathology of ALZHEIMER DISEASE. (Menkes, Textbook of Child Neurology, 5th ed, p213)		03.3260
Nerve Degeneration
Loss of functional activity and trophic degeneration of nerve axons and their terminal arborizations following the destruction of their cells of origin or interruption of their continuity with these cells. The pathology is characteristic of neurodegenerative diseases. Often the process of nerve degeneration is studied in research on neuroanatomical localization and correlation of the neurophysiology of neural pathways.		02.0810
Cognition Disorders
Disorders characterized by disturbances in mental processes related to learning, thinking, reasoning, and judgment.		015.034517
Age-Related Memory Disorders
[description not available]		02.7330
Memory Disorders
Disturbances in registering an impression, in the retention of an acquired impression, or in the recall of an impression. Memory impairments are associated with DEMENTIA; CRANIOCEREBRAL TRAUMA; ENCEPHALITIS; ALCOHOLISM (see also ALCOHOL AMNESTIC DISORDER); SCHIZOPHRENIA; and other conditions.		02.7330
Abnormality, Heart
[description not available]		02.7430
Infant, Newborn, Diseases
Diseases of newborn infants present at birth (congenital) or developing within the first month of birth. It does not include hereditary diseases not manifesting at birth or within the first 30 days of life nor does it include inborn errors of metabolism. Both HEREDITARY DISEASES and METABOLISM, INBORN ERRORS are available as general concepts.		04.96150
Heart Defects, Congenital
Developmental abnormalities involving structures of the heart. These defects are present at birth but may be discovered later in life.		02.7430
Kidney Diseases
Pathological processes of the KIDNEY or its component tissues.		04.65100
Cranial Nerve II Diseases
[description not available]		02.4720
Optic Nerve Diseases
Conditions which produce injury or dysfunction of the second cranial or optic nerve, which is generally considered a component of the central nervous system. Damage to optic nerve fibers may occur at or near their origin in the retina, at the optic disk, or in the nerve, optic chiasm, optic tract, or lateral geniculate nuclei. Clinical manifestations may include decreased visual acuity and contrast sensitivity, impaired color vision, and an afferent pupillary defect.		02.4720
Purine Pyrimidine Metabolism, Inborn Errors
[description not available]		04.0530
Emesis
[description not available]		012.722825
Vomiting
The forcible expulsion of the contents of the STOMACH through the MOUTH.		012.722825
Recrudescence
[description not available]		02.9940
Malignant Hypertension
[description not available]		02.110
Hypertension, Malignant
A condition of markedly elevated BLOOD PRESSURE with DIASTOLIC PRESSURE usually greater than 120 mm Hg. Malignant hypertension is characterized by widespread vascular damage, PAPILLEDEMA, retinopathy, HYPERTENSIVE ENCEPHALOPATHY, and renal dysfunction.		02.110
Blastocyst Disintegration
[description not available]		02.110
Delayed Effects, Prenatal Exposure
[description not available]		03.0110
Obesity
A status with BODY WEIGHT that is grossly above the recommended standards, usually due to accumulation of excess FATS in the body. The standards may vary with age, sex, genetic or cultural background. In the BODY MASS INDEX, a BMI greater than 30.0 kg/m2 is considered obese, and a BMI greater than 40.0 kg/m2 is considered morbidly obese (MORBID OBESITY).		05.5331
Benign Neoplasms, Brain
[description not available]		03.8721
Astrocytoma, Grade IV
[description not available]		02.110
Brain Neoplasms
Neoplasms of the intracranial components of the central nervous system, including the cerebral hemispheres, basal ganglia, hypothalamus, thalamus, brain stem, and cerebellum. Brain neoplasms are subdivided into primary (originating from brain tissue) and secondary (i.e., metastatic) forms. Primary neoplasms are subdivided into benign and malignant forms. In general, brain tumors may also be classified by age of onset, histologic type, or presenting location in the brain.		03.8721
Glioblastoma
A malignant form of astrocytoma histologically characterized by pleomorphism of cells, nuclear atypia, microhemorrhage, and necrosis. They may arise in any region of the central nervous system, with a predilection for the cerebral hemispheres, basal ganglia, and commissural pathways. Clinical presentation most frequently occurs in the fifth or sixth decade of life with focal neurologic signs or seizures.		02.110
Clerambault Syndrome
[description not available]		04.0150
Alcohol Drinking
Behaviors associated with the ingesting of ALCOHOLIC BEVERAGES, including social drinking.		05.2262
Diabetes Mellitus
A heterogeneous group of disorders characterized by HYPERGLYCEMIA and GLUCOSE INTOLERANCE.		05.4251
Dystonia
An attitude or posture due to the co-contraction of agonists and antagonist muscles in one region of the body. It most often affects the large axial muscles of the trunk and limb girdles. Conditions which feature persistent or recurrent episodes of dystonia as a primary manifestation of disease are referred to as DYSTONIC DISORDERS. (Adams et al., Principles of Neurology, 6th ed, p77)		09.0350
Encephalomyopathies, Mitochondrial
[description not available]		05.1460
Asymptomatic Colonization
[description not available]		02.1110
Swine Diseases
Diseases of domestic swine and of the wild boar of the genus Sus.		02.4320
Bilophila Infections
[description not available]		02.1110
Grippe
[description not available]		03.5111
Influenza, Human
An acute viral infection in humans involving the respiratory tract. It is marked by inflammation of the NASAL MUCOSA; the PHARYNX; and conjunctiva, and by headache and severe, often generalized, myalgia.		03.5111
Myeloproliferative Disorders
Conditions which cause proliferation of hemopoietically active tissue or of tissue which has embryonic hemopoietic potential. They all involve dysregulation of multipotent MYELOID PROGENITOR CELLS, most often caused by a mutation in the JAK2 PROTEIN TYROSINE KINASE.		02.4920
Autoimmune Disease
[description not available]		03.6630
Autoimmune Diseases
Disorders that are characterized by the production of antibodies that react with host tissues or immune effector cells that are autoreactive to endogenous peptides.		03.6630
Asymptomatic Conditions
[description not available]		02.5320
Metabolic Acidosis
[description not available]		07.39520
Acidosis
A pathologic condition of acid accumulation or depletion of base in the body. The two main types are RESPIRATORY ACIDOSIS and metabolic acidosis, due to metabolic acid build up.		07.39520
Age-Related Osteoporosis
[description not available]		02.9640
Osteoporosis
Reduction of bone mass without alteration in the composition of bone, leading to fractures. Primary osteoporosis can be of two major types: postmenopausal osteoporosis (OSTEOPOROSIS, POSTMENOPAUSAL) and age-related or senile osteoporosis.		02.9640
Plasmodium falciparum Malaria
[description not available]		02.1110
Malaria, Falciparum
Malaria caused by PLASMODIUM FALCIPARUM. This is the severest form of malaria and is associated with the highest levels of parasites in the blood. This disease is characterized by irregularly recurring febrile paroxysms that in extreme cases occur with acute cerebral, renal, or gastrointestinal manifestations.		02.1110
Atherogenesis
[description not available]		02.1110
Atherosclerosis
A thickening and loss of elasticity of the walls of ARTERIES that occurs with formation of ATHEROSCLEROTIC PLAQUES within the ARTERIAL INTIMA.		02.1110
Clinically Isolated CNS Demyelinating Syndrome
[description not available]		04.0450
Demyelinating Diseases
Diseases characterized by loss or dysfunction of myelin in the central or peripheral nervous system.		04.0450
Diseases, Occupational
[description not available]		02.1310
Diseases, Metabolic
[description not available]		05.5461
Amino Acid Transport Disorders, Inborn
Disorders characterized by defective transport of amino acids across cell membranes. These include deficits in transport across brush-border epithelial cell membranes of the small intestine (MICROVILLI) and KIDNEY TUBULES; transport across the basolateral membrane; and transport across the membranes of intracellular organelles. (From Nippon Rinsho 1992 Jul;50(7):1587-92)		02.1310
Metabolic Diseases
Generic term for diseases caused by an abnormal metabolic process. It can be congenital due to inherited enzyme abnormality (METABOLISM, INBORN ERRORS) or acquired due to disease of an endocrine organ or failure of a metabolically important organ such as the liver. (Stedman, 26th ed)		05.5461
Adenocarcinoma, Basal Cell
[description not available]		03.5111
Cancer of Rectum
[description not available]		03.8521
Adenocarcinoma
A malignant epithelial tumor with a glandular organization.		03.5111
Rectal Neoplasms
Tumors or cancer of the RECTUM.		03.8521
Cardiomyopathies, Primary
[description not available]		02.7730
Cardiomyopathies
A group of diseases in which the dominant feature is the involvement of the CARDIAC MUSCLE itself. Cardiomyopathies are classified according to their predominant pathophysiological features (DILATED CARDIOMYOPATHY; HYPERTROPHIC CARDIOMYOPATHY; RESTRICTIVE CARDIOMYOPATHY) or their etiological/pathological factors (CARDIOMYOPATHY, ALCOHOLIC; ENDOCARDIAL FIBROELASTOSIS).		02.7730
Absence Seizure
[description not available]		04.17160
Seizures
Clinical or subclinical disturbances of cortical function due to a sudden, abnormal, excessive, and disorganized discharge of brain cells. Clinical manifestations include abnormal motor, sensory and psychic phenomena. Recurrent seizures are usually referred to as EPILEPSY or seizure disorder.		09.17160
Nasopharyngeal Carcinoma
A carcinoma that originates in the EPITHELIUM of the NASOPHARYNX and includes four subtypes: keratinizing squamous cell, non-keratinizing, basaloid squamous cell, and PAPILLARY ADENOCARCINOMA. It is most prevalent in Southeast Asian populations and is associated with EPSTEIN-BARR VIRUS INFECTIONS. Somatic mutations associated with this cancer have been identified in NPCR, BAP1, UBAP1, ERBB2, ERBB3, MLL2, PIK3CA, KRAS, NRAS, and ARID1A genes.		02.1310
Carcinoma, Anaplastic
[description not available]		02.3820
Carcinoma, Epidermoid
[description not available]		02.1310
Cancer of Nasopharynx
[description not available]		02.1310
Carcinoma
A malignant neoplasm made up of epithelial cells tending to infiltrate the surrounding tissues and give rise to metastases. It is a histological type of neoplasm and not a synonym for cancer.		02.3820
Carcinoma, Squamous Cell
A carcinoma derived from stratified SQUAMOUS EPITHELIAL CELLS. It may also occur in sites where glandular or columnar epithelium is normally present. (From Stedman, 25th ed)		02.1310
Nasopharyngeal Neoplasms
Tumors or cancer of the NASOPHARYNX.		02.1310
Abnormalities, Multiple
Congenital abnormalities that affect more than one organ or body structure.		03.6530
Infectious Diseases
[description not available]		02.1510
Failure to Thrive
A condition of substandard growth or diminished capacity to maintain normal function.		03.2660
Hypogammaglobulinemia
[description not available]		02.1510
Agammaglobulinemia
An immunologic deficiency state characterized by an extremely low level of generally all classes of gamma-globulin in the blood.		02.1510
Communicable Diseases
An illness caused by an infectious agent or its toxins that occurs through the direct or indirect transmission of the infectious agent or its products from an infected individual or via an animal, vector or the inanimate environment to a susceptible animal or human host.		02.1510
Acrania
[description not available]		07.6291
Neural Tube Defects
Congenital malformations of the central nervous system and adjacent structures related to defective neural tube closure during the first trimester of pregnancy generally occurring between days 18-29 of gestation. Ectodermal and mesodermal malformations (mainly involving the skull and vertebrae) may occur as a result of defects of neural tube closure. (From Joynt, Clinical Neurology, 1992, Ch55, pp31-41)		07.6291
Abortion, Recurrent
[description not available]		02.0410
Abortion, Habitual
Three or more consecutive spontaneous abortions.		02.0410
Thromboembolism
Obstruction of a blood vessel (embolism) by a blood clot (THROMBUS) in the blood stream.		02.0410
Deficiency, Vitamin B
[description not available]		05.0770
Vitamin B Deficiency
A condition due to deficiency in any member of the VITAMIN B COMPLEX. These B vitamins are water-soluble and must be obtained from the diet because they are easily lost in the urine. Unlike the lipid-soluble vitamins, they cannot be stored in the body fat.		05.0770
Chromosome Deletion
Actual loss of portion of a chromosome.		02.4620
Abnormalities, Teeth
[description not available]		03.3720
Infections, Helicobacter
[description not available]		02.9440
Helicobacter Infections
Infections with organisms of the genus HELICOBACTER, particularly, in humans, HELICOBACTER PYLORI. The clinical manifestations are focused in the stomach, usually the gastric mucosa and antrum, and the upper duodenum. This infection plays a major role in the pathogenesis of type B gastritis and peptic ulcer disease.		02.9440
Scotoma
A localized defect in the visual field bordered by an area of normal vision. This occurs with a variety of EYE DISEASES (e.g., RETINAL DISEASES and GLAUCOMA); OPTIC NERVE DISEASES, and other conditions.		02.0510
Weight Gain
Increase in BODY WEIGHT over existing weight.		03.2460
Cerebral Ischemia
[description not available]		07.4220
Brain Ischemia
Localized reduction of blood flow to brain tissue due to arterial obstruction or systemic hypoperfusion. This frequently occurs in conjunction with brain hypoxia (HYPOXIA, BRAIN). Prolonged ischemia is associated with BRAIN INFARCTION.		02.4220
Arteriosclerosis, Coronary
[description not available]		02.0510
Coronary Artery Disease
Pathological processes of CORONARY ARTERIES that may derive from a congenital abnormality, atherosclerotic, or non-atherosclerotic cause.		07.0510
Cancer of Prostate
[description not available]		02.0510
Prostatic Neoplasms
Tumors or cancer of the PROSTATE.		02.0510
Granuloma, Hodgkin
[description not available]		02.0510
Diffuse Mixed Small and Large Cell Lymphoma
[description not available]		02.0510
Hodgkin Disease
A malignant disease characterized by progressive enlargement of the lymph nodes, spleen, and general lymphoid tissue. In the classical variant, giant usually multinucleate Hodgkin's and REED-STERNBERG CELLS are present; in the nodular lymphocyte predominant variant, lymphocytic and histiocytic cells are seen.		02.0510
Lymphoma, Non-Hodgkin
Any of a group of malignant tumors of lymphoid tissue that differ from HODGKIN DISEASE, being more heterogeneous with respect to malignant cell lineage, clinical course, prognosis, and therapy. The only common feature among these tumors is the absence of giant REED-STERNBERG CELLS, a characteristic of Hodgkin's disease.		02.0510
Mucositis
An INFLAMMATION of the MUCOSA with burning or tingling sensation. It is characterized by atrophy of the squamous EPITHELIUM, vascular damage, inflammatory infiltration, and ulceration. It usually occurs at the mucous lining of the MOUTH, the GASTROINTESTINAL TRACT or the airway due to chemical irritations, CHEMOTHERAPY, or radiation therapy (RADIOTHERAPY).		02.0510
Hyper IgD Syndrome
[description not available]		02.0510
Mevalonate Kinase Deficiency
Autosomal recessive disorder caused by mutations in the mevalonate kinase gene. Because of the mutations cholesterol biosynthesis is disrupted and MEVALONIC ACID accumulates. It is characterized by a range of symptoms, including dysmorphic FACIES, psychomotor retardation, CATARACT, hepatosplenomegaly, CEREBELLAR ATAXIA, elevated IMMUNOGLOBULIN D, and recurrent febrile crises with FEVER; LYMPHADENOPATHY; ARTHRALGIA; EDEMA; and rash.		02.0510
Encephalomyelitis, Subacute Necrotizing
[description not available]		03.6430
Leigh Disease
A group of metabolic disorders primarily of infancy characterized by the subacute onset of psychomotor retardation, hypotonia, ataxia, weakness, vision loss, eye movement abnormalities, seizures, dysphagia, and lactic acidosis. Pathological features include spongy degeneration of the neuropile of the basal ganglia, thalamus, brain stem, and spinal cord. Patterns of inheritance include X-linked recessive, autosomal recessive, and mitochondrial. Leigh disease has been associated with mutations in genes for the PYRUVATE DEHYDROGENASE COMPLEX; CYTOCHROME-C OXIDASE; ATP synthase subunit 6; and subunits of mitochondrial complex I. (From Menkes, Textbook of Child Neurology, 5th ed, p850).		03.6430
Uremia
A clinical syndrome associated with the retention of renal waste products or uremic toxins in the blood. It is usually the result of RENAL INSUFFICIENCY. Most uremic toxins are end products of protein or nitrogen CATABOLISM, such as UREA or CREATININE. Severe uremia can lead to multiple organ dysfunctions with a constellation of symptoms.		08.2360
Pancytopenia
Deficiency of all three cell elements of the blood, erythrocytes, leukocytes and platelets.		07.6730
Cochlear Hearing Loss
[description not available]		02.0510
Hearing Loss, Sensorineural
Hearing loss resulting from damage to the COCHLEA and the sensorineural elements which lie internally beyond the oval and round windows. These elements include the AUDITORY NERVE and its connections in the BRAINSTEM.		02.0510
Cryptogenic Infantile Spasms
[description not available]		02.4520
Spasms, Infantile
An epileptic syndrome characterized by the triad of infantile spasms, hypsarrhythmia, and arrest of psychomotor development at seizure onset. The majority present between 3-12 months of age, with spasms consisting of combinations of brief flexor or extensor movements of the head, trunk, and limbs. The condition is divided into two forms: cryptogenic (idiopathic) and symptomatic (secondary to a known disease process such as intrauterine infections; nervous system abnormalities; BRAIN DISEASES, METABOLIC, INBORN; prematurity; perinatal asphyxia; TUBEROUS SCLEROSIS; etc.). (From Menkes, Textbook of Child Neurology, 5th ed, pp744-8)		02.4520
Cardiac Diseases
[description not available]		02.0510
Heart Diseases
Pathological conditions involving the HEART including its structural and functional abnormalities.		02.0510
Genetic Non-Disjunction
[description not available]		02.0510
Aneuploid
[description not available]		02.0510
Chromosomal Triplication
[description not available]		02.4120
48,XXYY Syndrome
[description not available]		02.0510
Abnormalities, Sex Chromosome
[description not available]		02.0510
Klinefelter Syndrome
A form of male HYPOGONADISM, characterized by the presence of an extra X CHROMOSOME, small TESTES, seminiferous tubule dysgenesis, elevated levels of GONADOTROPINS, low serum TESTOSTERONE, underdeveloped secondary sex characteristics, and male infertility (INFERTILITY, MALE). Patients tend to have long legs and a slim, tall stature. GYNECOMASTIA is present in many of the patients. The classic form has the karyotype 47,XXY. Several karyotype variants include 48,XXYY; 48,XXXY; 49,XXXXY, and mosaic patterns ( 46,XY/47,XXY; 47,XXY/48,XXXY, etc.).		02.0510
Acute Disease
Disease having a short and relatively severe course.		04.76120
Electron Transfer Flavoprotein Alpha Subunit Deficiency
[description not available]		02.0410
Acrodermatitis
Inflammation involving the skin of the extremities, especially the hands and feet. Several forms are known, some idiopathic and some hereditary. The infantile form is called Gianotti-Crosti syndrome.		08.8340
BCKD Deficiency
[description not available]		06.75230
Maple Syrup Urine Disease
An autosomal recessive inherited disorder with multiple forms of phenotypic expression, caused by a defect in the oxidative decarboxylation of branched-chain amino acids (AMINO ACIDS, BRANCHED-CHAIN). These metabolites accumulate in body fluids and render a maple syrup odor. The disease is divided into classic, intermediate, intermittent, and thiamine responsive subtypes. The classic form presents in the first week of life with ketoacidosis, hypoglycemia, emesis, neonatal seizures, and hypertonia. The intermediate and intermittent forms present in childhood or later with acute episodes of ataxia and vomiting. (From Adams et al., Principles of Neurology, 6th ed, p936)		011.75230
Abortion, Tubal
[description not available]		02.0510
Abortion, Spontaneous
Expulsion of the product of FERTILIZATION before completing the term of GESTATION and without deliberate interference.		02.0510
Necrosis
The death of cells in an organ or tissue due to disease, injury or failure of the blood supply.		04.0730
Genetic Diseases
[description not available]		03.8240
Genetic Diseases, Inborn
Diseases that are caused by genetic mutations present during embryo or fetal development, although they may be observed later in life. The mutations may be inherited from a parent's genome or they may be acquired in utero.		03.8240
MODS
[description not available]		02.0510
Multiple Organ Failure
A progressive condition usually characterized by combined failure of several organs such as the lungs, liver, kidney, along with some clotting mechanisms, usually postinjury or postoperative.		02.0510
HbS Disease
[description not available]		02.7130
Experimental Lung Inflammation
Inflammation of any part, segment or lobe, of the lung parenchyma.		02.0510
Acute Hemolytic Transfusion Reaction
[description not available]		02.4120
SC Disease
[description not available]		02.0510
Anemia, Sickle Cell
A disease characterized by chronic hemolytic anemia, episodic painful crises, and pathologic involvement of many organs. It is the clinical expression of homozygosity for hemoglobin S.		02.7130
Hemoglobin SC Disease
One of the sickle cell disorders characterized by the presence of both hemoglobin S and hemoglobin C. It is similar to, but less severe than sickle cell anemia.		02.0510
Pneumonia
Infection of the lung often accompanied by inflammation.		02.0510
Iron Overload
An excessive accumulation of iron in the body due to a greater than normal absorption of iron from the gastrointestinal tract or from parenteral injection. This may arise from idiopathic hemochromatosis, excessive iron intake, chronic alcoholism, certain types of refractory anemia, or transfusional hemosiderosis. (From Churchill's Illustrated Medical Dictionary, 1989)		02.0510
Transfusion Reaction
Complications of BLOOD TRANSFUSION. Included adverse reactions are common allergic and febrile reactions; hemolytic (delayed and acute) reactions; and other non-hemolytic adverse reactions such as infections and adverse immune reactions related to immunocompatibility.		02.4120
Anemia
A reduction in the number of circulating ERYTHROCYTES or in the quantity of HEMOGLOBIN.		05.95142
Craniofacial Abnormalities
Congenital structural deformities, malformations, or other abnormalities of the cranium and facial bones.		02.9710
CACH Syndrome
[description not available]		02.0510
ANS (Autonomic Nervous System) Diseases
[description not available]		02.0510
Depression, Endogenous
[description not available]		02.0510
Depressive Disorder
An affective disorder manifested by either a dysphoric mood or loss of interest or pleasure in usual activities. The mood disturbance is prominent and relatively persistent.		02.0510
ALS - Amyotrophic Lateral Sclerosis
[description not available]		02.0510
Ophthalmoplegia, Progressive Supranuclear
[description not available]		02.0510
Amyotrophic Lateral Sclerosis
A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94)		02.0510
Supranuclear Palsy, Progressive
A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7)		02.0510
Deficiency Diseases
A condition produced by dietary or metabolic deficiency. The term includes all diseases caused by an insufficient supply of essential nutrients, i.e., protein (or amino acids), vitamins, and minerals. It also includes an inadequacy of calories. (From Dorland, 27th ed; Stedman, 25th ed)		02.0510
Amblyopia, Developmental
[description not available]		02.0510
Breathlessness
[description not available]		02.4620
Amblyopia
A nonspecific term referring to impaired vision. Major subcategories include stimulus deprivation-induced amblyopia and toxic amblyopia. Stimulus deprivation-induced amblyopia is a developmental disorder of the visual cortex. A discrepancy between visual information received by the visual cortex from each eye results in abnormal cortical development. STRABISMUS and REFRACTIVE ERRORS may cause this condition. Toxic amblyopia is a disorder of the OPTIC NERVE which is associated with ALCOHOLISM, tobacco SMOKING, and other toxins and as an adverse effect of the use of some medications.		07.0510
Anorexia
The lack or loss of APPETITE accompanied by an aversion to food and the inability to eat. It is the defining characteristic of the disorder ANOREXIA NERVOSA.		02.420
Dyspnea
Difficult or labored breathing.		02.4620
Ataxia of Gait
[description not available]		02.9810
Postgastrectomy Syndromes
Sequelae of gastrectomy from the second week after operation on. Include recurrent or anastomotic ulcer, postprandial syndromes (DUMPING SYNDROME and late postprandial hypoglycemia), disordered bowel action, and nutritional deficiencies.		03.3420
Lassitude
[description not available]		02.0510
Fatigue
The state of weariness following a period of exertion, mental or physical, characterized by a decreased capacity for work and reduced efficiency to respond to stimuli.		02.0510
Peripheral Arterial Diseases
[description not available]		02.0610
Peripheral Arterial Disease
Lack of perfusion in the EXTREMITIES resulting from atherosclerosis. It is characterized by INTERMITTENT CLAUDICATION, and an ANKLE BRACHIAL INDEX of 0.9 or less.		02.0610
Heart Disease, Ischemic
[description not available]		02.0610
Systolic Heart Failure
[description not available]		02.0610
Myocardial Ischemia
A disorder of cardiac function caused by insufficient blood flow to the muscle tissue of the heart. The decreased blood flow may be due to narrowing of the coronary arteries (CORONARY ARTERY DISEASE), to obstruction by a thrombus (CORONARY THROMBOSIS), or less commonly, to diffuse narrowing of arterioles and other small vessels within the heart. Severe interruption of the blood supply to the myocardial tissue may result in necrosis of cardiac muscle (MYOCARDIAL INFARCTION).		02.0610
Heart Failure, Systolic
Heart failure caused by abnormal myocardial contraction during SYSTOLE leading to defective cardiac emptying.		02.0610
Degenerative Diseases, Central Nervous System
[description not available]		03.8420
Neurodegenerative Diseases
Hereditary and sporadic conditions which are characterized by progressive nervous system dysfunction. These disorders are often associated with atrophy of the affected central or peripheral nervous system structures.		03.8420
Hypermelanosis
[description not available]		02.0610
Hyperpigmentation
Excessive pigmentation of the skin, usually as a result of increased epidermal or dermal melanin pigmentation, hypermelanosis. Hyperpigmentation can be localized or generalized. The condition may arise from exposure to light, chemicals or other substances, or from a primary metabolic imbalance.		02.0610
Blood Clot
[description not available]		02.4420
Thrombosis
Formation and development of a thrombus or blood clot in the blood vessel.		02.4420
Diarrhea
An increased liquidity or decreased consistency of FECES, such as running stool. Fecal consistency is related to the ratio of water-holding capacity of insoluble solids to total water, rather than the amount of water present. Diarrhea is not hyperdefecation or increased fecal weight.		04.7421
Abdominal Epilepsy
[description not available]		02.0610
Epilepsies, Partial
Conditions characterized by recurrent paroxysmal neuronal discharges which arise from a focal region of the brain. Partial seizures are divided into simple and complex, depending on whether consciousness is unaltered (simple partial seizure) or disturbed (complex partial seizure). Both types may feature a wide variety of motor, sensory, and autonomic symptoms. Partial seizures may be classified by associated clinical features or anatomic location of the seizure focus. A secondary generalized seizure refers to a partial seizure that spreads to involve the brain diffusely. (From Adams et al., Principles of Neurology, 6th ed, pp317)		02.0610
Deficiency, Pyridoxine
[description not available]		06.4771
Colitis
Inflammation of the COLON section of the large intestine (INTESTINE, LARGE), usually with symptoms such as DIARRHEA (often with blood and mucus), ABDOMINAL PAIN, and FEVER.		02.0610
Chronic Illness
[description not available]		03.3970
Chronic Disease
Diseases which have one or more of the following characteristics: they are permanent, leave residual disability, are caused by nonreversible pathological alteration, require special training of the patient for rehabilitation, or may be expected to require a long period of supervision, observation, or care (Dictionary of Health Services Management, 2d ed). For epidemiological studies chronic disease often includes HEART DISEASES; STROKE; CANCER; and diabetes (DIABETES MELLITUS, TYPE 2).		03.3970
Lactic Acidosis
[description not available]		02.4220
Acidosis, Lactic
Acidosis caused by accumulation of lactic acid more rapidly than it can be metabolized. It may occur spontaneously or in association with diseases such as DIABETES MELLITUS; LEUKEMIA; or LIVER FAILURE.		02.4220
Rubeola
[description not available]		02.4820
Measles
A highly contagious infectious disease caused by MORBILLIVIRUS, common among children but also seen in the nonimmune of any age, in which the virus enters the respiratory tract via droplet nuclei and multiplies in the epithelial cells, spreading throughout the MONONUCLEAR PHAGOCYTE SYSTEM.		02.4820
Amaurosis
[description not available]		02.0710
Blindness
The inability to see or the loss or absence of perception of visual stimuli. This condition may be the result of EYE DISEASES; OPTIC NERVE DISEASES; OPTIC CHIASM diseases; or BRAIN DISEASES affecting the VISUAL PATHWAYS or OCCIPITAL LOBE.		02.0710
Day Blindness
[description not available]		03.6930
Cerebral Palsy, Athetoid
[description not available]		02.0710
Cerebral Palsy
A heterogeneous group of nonprogressive motor disorders caused by chronic brain injuries that originate in the prenatal period, perinatal period, or first few years of life. The four major subtypes are spastic, athetoid, ataxic, and mixed cerebral palsy, with spastic forms being the most common. The motor disorder may range from difficulties with fine motor control to severe spasticity (see MUSCLE SPASTICITY) in all limbs. Spastic diplegia (Little disease) is the most common subtype, and is characterized by spasticity that is more prominent in the legs than in the arms. Pathologically, this condition may be associated with LEUKOMALACIA, PERIVENTRICULAR. (From Dev Med Child Neurol 1998 Aug;40(8):520-7)		02.0710
Dermatoses
[description not available]		02.6930
Infant Malnutrition
Malnutrition, occurring in infants ages 1 month to 24 months, which is due to insufficient intake of food, dietary nutrients, or a pathophysiologic condition which prevents the absorption and utilization of food. Growth and development are markedly affected.		03.6130
Skin Diseases
Diseases involving the DERMIS or EPIDERMIS.		02.6930
Choline Deficiency
A condition produced by a deficiency of CHOLINE in animals. Choline is known as a lipotropic agent because it has been shown to promote the transport of excess fat from the liver under certain conditions in laboratory animals. Combined deficiency of choline (included in the B vitamin complex) and all other methyl group donors causes liver cirrhosis in some animals. Unlike compounds normally considered as vitamins, choline does not serve as a cofactor in enzymatic reactions. (From Saunders Dictionary & Encyclopedia of Laboratory Medicine and Technology, 1984)		02.0810
Abnormalities, Autosome
[description not available]		03.150
Isolated Non-compaction of the Ventricular Myocardium
[description not available]		02.0710
Branch Retinal Artery Occlusion
[description not available]		02.0710
Retinal Artery Occlusion
Sudden ISCHEMIA in the RETINA due to blocked blood flow through the CENTRAL RETINAL ARTERY or its branches leading to sudden complete or partial loss of vision, respectively, in the eye.		02.0710
Alcohol Abuse
[description not available]		02.7230
Liver Dysfunction
[description not available]		03.150
Alcoholism
A primary, chronic disease with genetic, psychosocial, and environmental factors influencing its development and manifestations. The disease is often progressive and fatal. It is characterized by impaired control over drinking, preoccupation with the drug alcohol, use of alcohol despite adverse consequences, and distortions in thinking, most notably denial. Each of these symptoms may be continuous or periodic. (Morse & Flavin for the Joint Commission of the National Council on Alcoholism and Drug Dependence and the American Society of Addiction Medicine to Study the Definition and Criteria for the Diagnosis of Alcoholism: in JAMA 1992;268:1012-4)		02.7230
Liver Diseases
Pathological processes of the LIVER.		03.150
Child Development Deviations
[description not available]		012.522216
Developmental Disabilities
Disorders in which there is a delay in development based on that expected for a given age level or stage of development. These impairments or disabilities originate before age 18, may be expected to continue indefinitely, and constitute a substantial impairment. Biological and nonbiological factors are involved in these disorders. (From American Psychiatric Glossary, 6th ed)		012.522216
Child Malnutrition
Malnutrition occurring in children ages 2 to 12 years, which is due to insufficient intake of food, dietary nutrients, or a pathophysiologic condition which prevents the absorption and utilization of food. Growth and development are markedly affected.		0310
Experimental Neoplasms
[description not available]		02.0810
Hereditary Optic Neuroretinopathy
[description not available]		02.110
Optic Atrophy, Hereditary, Leber
A maternally linked genetic disorder that presents in mid-life as acute or subacute central vision loss leading to central scotoma and blindness. The disease has been associated with missense mutations in the mtDNA, in genes for Complex I, III, and IV polypeptides, that can act autonomously or in association with each other to cause the disease. (from Online Mendelian Inheritance in Man, http://www.ncbi.nlm.nih.gov/Omim/, MIM#535000 (April 17, 2001))		02.110
Microcephaly
A congenital abnormality in which the CEREBRUM is underdeveloped, the fontanels close prematurely, and, as a result, the head is small. (Desk Reference for Neuroscience, 2nd ed.)		07.420
Hematuria
Presence of blood in the urine.		02.0110
Extravascular Hemolysis
[description not available]		02.0110
Gasser Syndrome
[description not available]		04.7770
Hemolysis
The destruction of ERYTHROCYTES by many different causal agents such as antibodies, bacteria, chemicals, temperature, and changes in tonicity.		02.0110
Hemolytic-Uremic Syndrome
A syndrome that is associated with microvascular diseases of the KIDNEY, such as RENAL CORTICAL NECROSIS. It is characterized by hemolytic anemia (ANEMIA, HEMOLYTIC); THROMBOCYTOPENIA; and ACUTE RENAL FAILURE.		04.7770
Behavior Disorders
[description not available]		08.12121
Mental Disorders
Psychiatric illness or diseases manifested by breakdowns in the adaptational process expressed primarily as abnormalities of thought, feeling, and behavior producing either distress or impairment of function.		08.12121
G(M2) Gangliosidoses
[description not available]		02.9210
Aura
[description not available]		03.8240
Classic Globoid Cell Leukodystrophy
[description not available]		02.9210
Adult Neuronal Ceroid Lipofuscinosis
[description not available]		03.820
Niemann-Pick Disease
[description not available]		02.9210
Epilepsy
A disorder characterized by recurrent episodes of paroxysmal brain dysfunction due to a sudden, disorderly, and excessive neuronal discharge. Epilepsy classification systems are generally based upon: (1) clinical features of the seizure episodes (e.g., motor seizure), (2) etiology (e.g., post-traumatic), (3) anatomic site of seizure origin (e.g., frontal lobe seizure), (4) tendency to spread to other structures in the brain, and (5) temporal patterns (e.g., nocturnal epilepsy). (From Adams et al., Principles of Neurology, 6th ed, p313)		03.8240
Leukodystrophy, Globoid Cell
An autosomal recessive metabolic disorder caused by a deficiency of GALACTOSYLCERAMIDASE leading to intralysosomal accumulation of galactolipids such as GALACTOSYLCERAMIDES and PSYCHOSINE. It is characterized by demyelination associated with large multinucleated globoid cells, predominantly involving the white matter of the central nervous system. The loss of MYELIN disrupts normal conduction of nerve impulses.		02.9210
Neuronal Ceroid-Lipofuscinoses
A group of severe neurodegenerative diseases characterized by intracellular accumulation of autofluorescent wax-like lipid materials (CEROID; LIPOFUSCIN) in neurons. There are several subtypes based on mutations of the various genes, time of disease onset, and severity of the neurological defects such as progressive DEMENTIA; SEIZURES; and visual failure.		03.820
Niemann-Pick Diseases
A group of autosomal recessive disorders in which harmful quantities of lipids accumulate in the viscera and the central nervous system. They can be caused by deficiencies of enzyme activities (SPHINGOMYELIN PHOSPHODIESTERASE) or defects in intracellular transport, resulting in the accumulation of SPHINGOMYELINS and CHOLESTEROL. There are various subtypes based on their clinical and genetic differences.		02.9210
Gangliosidoses, GM2
A group of recessively inherited diseases characterized by the intralysosomal accumulation of G(M2) GANGLIOSIDE in the neuronal cells. Subtypes include mutations of enzymes in the BETA-N-ACETYLHEXOSAMINIDASES system or G(M2) ACTIVATOR PROTEIN leading to disruption of normal degradation of GANGLIOSIDES, a subclass of ACIDIC GLYCOSPHINGOLIPIDS.		02.9210
Left Ventricular Hypertrophy
[description not available]		02.0110
Hypertrophy, Left Ventricular
Enlargement of the LEFT VENTRICLE of the heart. This increase in ventricular mass is attributed to sustained abnormal pressure or volume loads and is a contributor to cardiovascular morbidity and mortality.		02.0110
Anoxemia
[description not available]		02.0110
Hypoxia
Sub-optimal OXYGEN levels in the ambient air of living organisms.		02.0110
Rheumatoid Arthritis
[description not available]		07.4120
Libman-Sacks Disease
[description not available]		02.0210
Psoriasis Arthropathica
[description not available]		02.0210
Arthritis, Rheumatoid
A chronic systemic disease, primarily of the joints, marked by inflammatory changes in the synovial membranes and articular structures, widespread fibrinoid degeneration of the collagen fibers in mesenchymal tissues, and by atrophy and rarefaction of bony structures. Etiology is unknown, but autoimmune mechanisms have been implicated.		02.4120
Lupus Erythematosus, Systemic
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.		02.0210
Arthritis, Psoriatic
A type of inflammatory arthritis associated with PSORIASIS, often involving the axial joints and the peripheral terminal interphalangeal joints. It is characterized by the presence of HLA-B27-associated SPONDYLARTHROPATHY, and the absence of rheumatoid factor.		02.0210
Disease
A definite pathologic process with a characteristic set of signs and symptoms. It may affect the whole body or any of its parts, and its etiology, pathology, and prognosis may be known or unknown.		02.9310
MS (Multiple Sclerosis)
[description not available]		02.6830
Multiple Sclerosis
An autoimmune disorder mainly affecting young adults and characterized by destruction of myelin in the central nervous system. Pathologic findings include multiple sharply demarcated areas of demyelination throughout the white matter of the central nervous system. Clinical manifestations include visual loss, extra-ocular movement disorders, paresthesias, loss of sensation, weakness, dysarthria, spasticity, ataxia, and bladder dysfunction. The usual pattern is one of recurrent attacks followed by partial recovery (see MULTIPLE SCLEROSIS, RELAPSING-REMITTING), but acute fulminating and chronic progressive forms (see MULTIPLE SCLEROSIS, CHRONIC PROGRESSIVE) also occur. (Adams et al., Principles of Neurology, 6th ed, p903)		07.6830
Tonsillitis
Inflammation of the tonsils, especially the PALATINE TONSILS but the ADENOIDS (pharyngeal tonsils) and lingual tonsils may also be involved. Tonsillitis usually is caused by bacterial infection. Tonsillitis may be acute, chronic, or recurrent.		02.0110
Cancer of Kidney
[description not available]		02.0110
Kidney Neoplasms
Tumors or cancers of the KIDNEY.		02.0110
Mouth Diseases
Diseases involving the MOUTH.		02.0110
Mucositis, Oral
[description not available]		02.0110
Stomatitis
INFLAMMATION of the soft tissues of the MOUTH, such as MUCOSA; PALATE; GINGIVA; and LIP.		02.0110
Complications, Hematologic Pregnancy
[description not available]		02.3620
Celiac Sprue
[description not available]		02.3620
Diverticula
[description not available]		01.9410
Bleeding
[description not available]		02.3520
Nutritional Disorders
[description not available]		01.9410
Hypoascorbemia
[description not available]		01.9410
Diaphragmatic Hernia
[description not available]		01.9410
Celiac Disease
A malabsorption syndrome that is precipitated by the ingestion of foods containing GLUTEN, such as wheat, rye, and barley. It is characterized by INFLAMMATION of the SMALL INTESTINE, loss of MICROVILLI structure, failed INTESTINAL ABSORPTION, and MALNUTRITION.		02.3620
Enteritis
Inflammation of any segment of the SMALL INTESTINE.		01.9410
Hemorrhage
Bleeding or escape of blood from a vessel.		02.3520
Nutrition Disorders
Disorders caused by nutritional imbalance, either overnutrition or undernutrition.		01.9410
Scurvy
An acquired blood vessel disorder caused by severe deficiency of vitamin C (ASCORBIC ACID) in the diet leading to defective collagen formation in small blood vessels. Scurvy is characterized by bleeding in any tissue, weakness, ANEMIA, spongy gums, and a brawny induration of the muscles of the calves and legs.		01.9410
Gastritis
Inflammation of the GASTRIC MUCOSA, a lesion observed in a number of unrelated disorders.		07.0110
Stunted Growth
[description not available]		03.150
Growth Disorders
Deviations from the average values for a specific age and sex in any or all of the following: height, weight, skeletal proportions, osseous development, or maturation of features. Included here are both acceleration and retardation of growth.		03.150
Hypovolemic
[description not available]		02.0210
Hematoma, Subdural
Accumulation of blood in the SUBDURAL SPACE between the DURA MATER and the arachnoidal layer of the MENINGES. This condition primarily occurs over the surface of a CEREBRAL HEMISPHERE, but may develop in the spinal canal (HEMATOMA, SUBDURAL, SPINAL). Subdural hematoma can be classified as the acute or the chronic form, with immediate or delayed symptom onset, respectively. Symptoms may include loss of consciousness, severe HEADACHE, and deteriorating mental status.		02.0210
Hypothermia, Accidental
[description not available]		02.0210
Hemorrhage, Retinal
[description not available]		02.0210
Hematoma
A collection of blood outside the BLOOD VESSELS. Hematoma can be localized in an organ, space, or tissue.		02.0210
Hypothermia
Lower than normal body temperature, especially in warm-blooded animals.		02.0210
Hypovolemia
An abnormally low volume of blood circulating through the body. It may result in hypovolemic shock (see SHOCK).		02.0210
Deficiency, Thiamine
[description not available]		03.2920
Thiamine Deficiency
A nutritional condition produced by a deficiency of THIAMINE in the diet, characterized by anorexia, irritability, and weight loss. Later, patients experience weakness, peripheral neuropathy, headache, and tachycardia. In addition to being caused by a poor diet, thiamine deficiency in the United States most commonly occurs as a result of alcoholism, since ethanol interferes with thiamine absorption. In countries relying on polished rice as a dietary staple, BERIBERI prevalence is very high. (From Cecil Textbook of Medicine, 19th ed, p1171)		03.2920
Vascular Diseases
Pathological processes involving any of the BLOOD VESSELS in the cardiac or peripheral circulation. They include diseases of ARTERIES; VEINS; and rest of the vasculature system in the body.		03.6130
Hemolysis, Elevated Liver Enzymes, Lowered Platelets
[description not available]		02.0210
Edema-Proteinuria-Hypertension Gestosis
[description not available]		02.0210
Placental Insufficiency
Failure of the PLACENTA to deliver an adequate supply of nutrients and OXYGEN to the FETUS.		02.0210
Pre-Eclampsia
A complication of PREGNANCY, characterized by a complex of symptoms including maternal HYPERTENSION and PROTEINURIA with or without pathological EDEMA. Symptoms may range between mild and severe. Pre-eclampsia usually occurs after the 20th week of gestation, but may develop before this time in the presence of trophoblastic disease.		02.0210
HELLP Syndrome
A syndrome of HEMOLYSIS, elevated liver ENZYMES, and low blood platelets count (THROMBOCYTOPENIA). HELLP syndrome is observed in pregnant women with PRE-ECLAMPSIA or ECLAMPSIA who also exhibit LIVER damage and abnormalities in BLOOD COAGULATION.		02.0210
Refractory Anemia
[description not available]		02.0210
Anemia, Sideroblastic
Anemia characterized by the presence of erythroblasts containing excessive deposits of iron in the marrow.		03.2920
Anemia, Refractory
A severe sometimes chronic anemia, usually macrocytic in type, that does not respond to ordinary antianemic therapy.		02.0210
Deficiency Disease, Ornithine Carbamoyltransferase
[description not available]		04.5190
Carboxylase Deficiency, Combined
[description not available]		03.5930
Ornithine Carbamoyltransferase Deficiency Disease
An inherited urea cycle disorder associated with deficiency of the enzyme ORNITHINE CARBAMOYLTRANSFERASE, transmitted as an X-linked trait and featuring elevations of amino acids and ammonia in the serum. Clinical features, which are more prominent in males, include seizures, behavioral alterations, episodic vomiting, lethargy, and coma. (Menkes, Textbook of Child Neurology, 5th ed, pp49-50)		04.5190
Diabetic Glomerulosclerosis
[description not available]		03.821
Diabetic Nephropathies
KIDNEY injuries associated with diabetes mellitus and affecting KIDNEY GLOMERULUS; ARTERIOLES; KIDNEY TUBULES; and the interstitium. Clinical signs include persistent PROTEINURIA, from microalbuminuria progressing to ALBUMINURIA of greater than 300 mg/24 h, leading to reduced GLOMERULAR FILTRATION RATE and END-STAGE RENAL DISEASE.		03.821
Leukoaraiosis
Non-specific white matter changes in the BRAIN, often seen after age 65. Changes include loss of AXONS; MYELIN pallor, GLIOSIS, loss of ependymal cells, and enlarged perivascular spaces. Leukoaraiosis is a risk factor for DEMENTIA and CEREBROVASCULAR DISORDERS.		02.0210
Bewilderment
[description not available]		02.0210
Nephritis
Inflammation of any part of the KIDNEY.		02.0210
Interstitial Nephritis
[description not available]		08.0850
Nephritis, Interstitial
Inflammation of the interstitial tissue of the kidney. This term is generally used for primary inflammation of KIDNEY TUBULES and/or surrounding interstitium. For primary inflammation of glomerular interstitium, see GLOMERULONEPHRITIS. Infiltration of the inflammatory cells into the interstitial compartment results in EDEMA, increased spaces between the tubules, and tubular renal dysfunction.		03.0850
Embryopathies
[description not available]		04.2770
Cyst
[description not available]		02.0210
Cystic Periventricular Leukomalacia
[description not available]		02.0210
Leukomalacia, Periventricular
Degeneration of white matter adjacent to the CEREBRAL VENTRICLES following cerebral hypoxia or BRAIN ISCHEMIA in neonates. The condition primarily affects white matter in the perfusion zone between superficial and deep branches of the MIDDLE CEREBRAL ARTERY. Clinical manifestations include VISION DISORDERS; CEREBRAL PALSY; PARAPLEGIA; SEIZURES; and cognitive disorders. (From Adams et al., Principles of Neurology, 6th ed, p1021; Joynt, Clinical Neurology, 1997, Ch4, pp30-1)		02.0210
Luft Disease
[description not available]		02.0210
Muscular Weakness
[description not available]		02.0210
Mitochondrial Myopathies
A group of muscle diseases associated with abnormal mitochondria function.		02.0210
Muscle Weakness
A vague complaint of debility, fatigue, or exhaustion attributable to weakness of various muscles. The weakness can be characterized as subacute or chronic, often progressive, and is a manifestation of many muscle and neuromuscular diseases. (From Wyngaarden et al., Cecil Textbook of Medicine, 19th ed, p2251)		02.0210
Erythrophagocytic Lymphohistiocytosis, Familial
[description not available]		02.0210
Lymphohistiocytosis, Hemophagocytic
A group of related disorders characterized by LYMPHOCYTOSIS; HISTIOCYTOSIS; and hemophagocytosis. The two major forms are familial and reactive.		02.0210
EBV Infections
[description not available]		02.0210
Cytomegalic Inclusion Disease
[description not available]		02.0210
Carbamoyl Phosphate Synthase (Ammonia) Deficiency Disease
[description not available]		02.4320
Cytomegalovirus Infections
Infection with CYTOMEGALOVIRUS, characterized by enlarged cells bearing intranuclear inclusions. Infection may be in almost any organ, but the salivary glands are the most common site in children, as are the lungs in adults.		02.0210
Epstein-Barr Virus Infections
Infection with human herpesvirus 4 (HERPESVIRUS 4, HUMAN); which may facilitate the development of various lymphoproliferative disorders. These include BURKITT LYMPHOMA (African type), INFECTIOUS MONONUCLEOSIS, and oral hairy leukoplakia (LEUKOPLAKIA, HAIRY).		02.0210
Carbamoyl-Phosphate Synthase I Deficiency Disease
A urea cycle disorder manifesting in infancy as lethargy, emesis, seizures, alterations of muscle tone, abnormal eye movements, and an elevation of serum ammonia. The disorder is caused by a reduction in the activity of hepatic mitochondrial CARBAMOYL-PHOSPHATE SYNTHASE (AMMONIA). (Menkes, Textbook of Child Neurology, 5th ed, pp50-1)		02.4320
Carotid Artery Narrowing
[description not available]		02.0210
Carotid Stenosis
Narrowing or stricture of any part of the CAROTID ARTERIES, most often due to atherosclerotic plaque formation. Ulcerations may form in atherosclerotic plaques and induce THROMBUS formation. Platelet or cholesterol emboli may arise from stenotic carotid lesions and induce a TRANSIENT ISCHEMIC ATTACK; CEREBROVASCULAR ACCIDENT; or temporary blindness (AMAUROSIS FUGAX). (From Adams et al., Principles of Neurology, 6th ed, pp 822-3)		02.0210
Developmental Psychomotor Disorders
[description not available]		02.4120
Deafness, Transitory
[description not available]		03.8221
Infection
[description not available]		02.4420
Infections
Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases.		02.4420
Hearing Loss
A general term for the complete or partial loss of the ability to hear from one or both ears.		08.8221
Anesthesia
A state characterized by loss of feeling or sensation. This depression of nerve function is usually the result of pharmacologic action and is induced to allow performance of surgery or other painful procedures.		02.0310
Cleft Spine
[description not available]		02.9410
Lipid Metabolism, Inborn Error
[description not available]		05.99120
Deaf Mutism
[description not available]		02.9510
Deafness
A general term for the complete loss of the ability to hear from both ears.		07.9510
Diseases in Twins
Disorders affecting TWINS, one or both, at any age.		02.3920
Coma
A profound state of unconsciousness associated with depressed cerebral activity from which the individual cannot be aroused. Coma generally occurs when there is dysfunction or injury involving both cerebral hemispheres or the brain stem RETICULAR FORMATION.		02.0310
Overweight
A status with BODY WEIGHT that is above certain standards. In the scale of BODY MASS INDEX, overweight is defined as having a BMI of 25.0-29.9 kg/m2. Overweight may or may not be due to increases in body fat (ADIPOSE TISSUE), hence overweight does not equal over fat.		03.4311
Testicular Diseases
Pathological processes of the TESTIS.		02.0310
Infections, Respiratory
[description not available]		02.9510
Respiratory Tract Infections
Invasion of the host RESPIRATORY SYSTEM by microorganisms, usually leading to pathological processes or diseases.		02.9510
HIV Coinfection
[description not available]		04.0931
Complications, Infectious Pregnancy
[description not available]		02.0310
HIV Infections
Includes the spectrum of human immunodeficiency virus infections that range from asymptomatic seropositivity, thru AIDS-related complex (ARC), to acquired immunodeficiency syndrome (AIDS).		04.0931
Acid-Base Imbalance
Disturbances in the ACID-BASE EQUILIBRIUM of the body.		02.0310
Marfan Syndrome, Type I
[description not available]		02.0310
Marfan Syndrome
An autosomal dominant disorder of CONNECTIVE TISSUE with abnormal features in the heart, the eye, and the skeleton. Cardiovascular manifestations include MITRAL VALVE PROLAPSE, dilation of the AORTA, and aortic dissection. Other features include lens displacement (ectopia lentis), disproportioned long limbs and enlarged DURA MATER (dural ectasia). Marfan syndrome (type 1) is associated with mutations in the gene encoding FIBRILLIN-1 (FBN1), a major element of extracellular microfibrils of connective tissue. Mutations in the gene encoding TYPE II TGF-BETA RECEPTOR (TGFBR2) are associated with Marfan syndrome type 2.		02.0310
Carcinoma, Non-Small Cell Lung
[description not available]		03.4211
Cancer of Lung
[description not available]		03.4211
Carcinoma, Non-Small-Cell Lung
A heterogeneous aggregate of at least three distinct histological types of lung cancer, including SQUAMOUS CELL CARCINOMA; ADENOCARCINOMA; and LARGE CELL CARCINOMA. They are dealt with collectively because of their shared treatment strategy.		03.4211
Lung Neoplasms
Tumors or cancer of the LUNG.		03.4211
Urinary Tract Diseases
[description not available]		03.4211
Dementia Praecox
[description not available]		02.0410
Schizophrenia
A severe emotional disorder of psychotic depth characteristically marked by a retreat from reality with delusion formation, HALLUCINATIONS, emotional disharmony, and regressive behavior.		07.0410
Hyperglycemia, Postprandial
Abnormally high BLOOD GLUCOSE level after a meal.		03.5630
Acetonemia
[description not available]		05.22120
Hyperglycemia
Abnormally high BLOOD GLUCOSE level.		08.5630
Methemoglobinemia
The presence of methemoglobin in the blood, resulting in cyanosis. A small amount of methemoglobin is present in the blood normally, but injury or toxic agents convert a larger proportion of hemoglobin into methemoglobin, which does not function reversibly as an oxygen carrier. Methemoglobinemia may be due to a defect in the enzyme NADH methemoglobin reductase (an autosomal recessive trait) or to an abnormality in hemoglobin M (an autosomal dominant trait). (Dorland, 27th ed)		02.8810
Ataxia with Lactic Acidosis 2
[description not available]		02.8810
Antibody Deficiency Syndrome
[description not available]		02.3720
Immunologic Deficiency Syndromes
Syndromes in which there is a deficiency or defect in the mechanisms of immunity, either cellular or humoral.		02.3720
Amino Acid Transport Disorder, Neutral
[description not available]		03.7840
Cerebellar Diseases
Diseases that affect the structure or function of the cerebellum. Cardinal manifestations of cerebellar dysfunction include dysmetria, GAIT ATAXIA, and MUSCLE HYPOTONIA.		01.9610
Bacterial Disease
[description not available]		01.9510
Bacterial Infections
Infections by bacteria, general or unspecified.		01.9510
Ovine Diseases
[description not available]		04.6161
Bovine Diseases
[description not available]		02.6630
Keratitis, Ulcerative
[description not available]		01.9610
Xerophthalmia
Dryness of the eye surfaces caused by deficiency of tears or conjunctival secretions. It may be associated with vitamin A deficiency, trauma, or any condition in which the eyelids do not close completely.		01.9610
Corneal Ulcer
Loss of epithelial tissue from the surface of the cornea due to progressive erosion and necrosis of the tissue; usually caused by bacterial, fungal, or viral infection.		01.9610
Myoclonic Jerk
[description not available]		01.9510
Eyelid Diseases
Diseases involving the EYELIDS.		01.9510
Di Guglielmo Disease
[description not available]		01.9810
Leukemia, Erythroblastic, Acute
A myeloproliferative disorder characterized by neoplastic proliferation of erythroblastic and myeloblastic elements with atypical erythroblasts and myeloblasts in the peripheral blood.		01.9810
Hemoglobinopathies
A group of inherited disorders characterized by structural alterations within the hemoglobin molecule.		01.9810
Cataract, Membranous
[description not available]		01.9810
Cataract
Partial or complete opacity on or in the lens or capsule of one or both eyes, impairing vision or causing blindness. The many kinds of cataract are classified by their morphology (size, shape, location) or etiology (cause and time of occurrence). (Dorland, 27th ed)		06.9810
Urinary Calculi
Low-density crystals or stones in any part of the URINARY TRACT. Their chemical compositions often include CALCIUM OXALATE, magnesium ammonium phosphate (struvite), CYSTINE, or URIC ACID.		01.9810
Fasting Hypoglycemia
HYPOGLYCEMIA expressed in the postabsorptive state, after prolonged FASTING, or an overnight fast.		04.0431
Hypoglycemia
A syndrome of abnormally low BLOOD GLUCOSE level. Clinical hypoglycemia has diverse etiologies. Severe hypoglycemia eventually lead to glucose deprivation of the CENTRAL NERVOUS SYSTEM resulting in HUNGER; SWEATING; PARESTHESIA; impaired mental function; SEIZURES; COMA; and even DEATH.		09.0431
Bacteremia
The presence of viable bacteria circulating in the blood. Fever, chills, tachycardia, and tachypnea are common acute manifestations of bacteremia. The majority of cases are seen in already hospitalized patients, most of whom have underlying diseases or procedures which render their bloodstreams susceptible to invasion.		03.3711
Autosomal Chromosome Disorders
[description not available]		01.9810
Bladder Disorder, Neurogenic
[description not available]		01.9810
Dyskinesia Syndromes
[description not available]		01.9810
Urinary Bladder, Neurogenic
Dysfunction of the URINARY BLADDER due to disease of the central or peripheral nervous system pathways involved in the control of URINATION. This is often associated with SPINAL CORD DISEASES, but may also be caused by BRAIN DISEASES or PERIPHERAL NERVE DISEASES.		01.9810
Movement Disorders
Syndromes which feature DYSKINESIAS as a cardinal manifestation of the disease process. Included in this category are degenerative, hereditary, post-infectious, medication-induced, post-inflammatory, and post-traumatic conditions.		01.9810
Hyperlipemia
[description not available]		03.2820
Arteriosclerosis
Thickening and loss of elasticity of the walls of ARTERIES of all sizes. There are many forms classified by the types of lesions and arteries involved, such as ATHEROSCLEROSIS with fatty lesions in the ARTERIAL INTIMA of medium and large muscular arteries.		07.6830
Hyperlipidemias
Conditions with excess LIPIDS in the blood.		03.2820
Autoimmune Diabetes
[description not available]		02.3820
Diabetes Mellitus, Type 1
A subtype of DIABETES MELLITUS that is characterized by INSULIN deficiency. It is manifested by the sudden onset of severe HYPERGLYCEMIA, rapid progression to DIABETIC KETOACIDOSIS, and DEATH unless treated with insulin. The disease may occur at any age, but is most common in childhood or adolescence.		02.3820
Muscle Disorders
[description not available]		01.9810
Muscular Diseases
Acquired, familial, and congenital disorders of SKELETAL MUSCLE and SMOOTH MUSCLE.		01.9810
Anasarca
[description not available]		01.9810
Dermatitis
Any inflammation of the skin.		01.9810
Edema
Abnormal fluid accumulation in TISSUES or body cavities. Most cases of edema are present under the SKIN in SUBCUTANEOUS TISSUE.		01.9810
Erythema
Redness of the skin produced by congestion of the capillaries. This condition may result from a variety of disease processes.		01.9810
Complications of Diabetes Mellitus
[description not available]		01.9810
Femoral Fractures
Fractures of the femur.		01.9810
Fracture, Pathologic
[description not available]		01.9810
Candidiasis, Cutaneous
Candidiasis of the skin manifested as eczema-like lesions of the interdigital spaces, perleche, or chronic paronychia. (Dorland, 27th ed)		01.9810
Central Nervous System Disease
[description not available]		04.3830
Central Nervous System Diseases
Diseases of any component of the brain (including the cerebral hemispheres, diencephalon, brain stem, and cerebellum) or the spinal cord.		04.3830
Infections, Pseudomonas
[description not available]		01.9810
Ecthyma
An ulcerative pyoderma usually caused by group A beta-hemolytic streptococcal infection at the site of minor trauma. (Dorland, 27th ed)		06.9810
Pseudomonas Infections
Infections with bacteria of the genus PSEUDOMONAS.		01.9810
Acute Edematous Pancreatitis
[description not available]		01.9810
Pancreatitis
INFLAMMATION of the PANCREAS. Pancreatitis is classified as acute unless there are computed tomographic or endoscopic retrograde cholangiopancreatographic findings of CHRONIC PANCREATITIS (International Symposium on Acute Pancreatitis, Atlanta, 1992). The two most common forms of acute pancreatitis are ALCOHOLIC PANCREATITIS and gallstone pancreatitis.		01.9810
Blood Diseases
[description not available]		04.0530
Hematologic Diseases
Disorders of the blood and blood forming tissues.		04.0530
Basal Ganglia Diseases
Diseases of the BASAL GANGLIA including the PUTAMEN; GLOBUS PALLIDUS; claustrum; AMYGDALA; and CAUDATE NUCLEUS. DYSKINESIAS (most notably involuntary movements and alterations of the rate of movement) represent the primary clinical manifestations of these disorders. Common etiologies include CEREBROVASCULAR DISORDERS; NEURODEGENERATIVE DISEASES; and CRANIOCEREBRAL TRAUMA.		03.9950
Gastroduodenal Ulcer
[description not available]		01.9910
Peptic Ulcer
Ulcer that occurs in the regions of the GASTROINTESTINAL TRACT which come into contact with GASTRIC JUICE containing PEPSIN and GASTRIC ACID. It occurs when there are defects in the MUCOSA barrier. The common forms of peptic ulcers are associated with HELICOBACTER PYLORI and the consumption of nonsteroidal anti-inflammatory drugs (NSAIDS).		01.9910
Encephalomyelitis, Inflammatory
[description not available]		01.9910
Encephalomyelitis
A general term indicating inflammation of the BRAIN and SPINAL CORD, often used to indicate an infectious process, but also applicable to a variety of autoimmune and toxic-metabolic conditions. There is significant overlap regarding the usage of this term and ENCEPHALITIS in the literature.		01.9910
Atresia, Biliary
[description not available]		01.9910
Cerebro-Hepato-Renal Syndrome
[description not available]		01.9910
Biliary Atresia
Progressive destruction or the absence of all or part of the extrahepatic BILE DUCTS, resulting in the complete obstruction of BILE flow. Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE.		01.9910
Zellweger Syndrome
An autosomal recessive disorder due to defects in PEROXISOME biogenesis which involves more than 13 genes encoding peroxin proteins of the peroxisomal membrane and matrix. Zellweger syndrome is typically seen in the neonatal period with features such as dysmorphic skull; MUSCLE HYPOTONIA; SENSORINEURAL HEARING LOSS; visual compromise; SEIZURES; progressive degeneration of the KIDNEYS and the LIVER. Zellweger-like syndrome refers to phenotypes resembling the neonatal Zellweger syndrome but seen in children or adults with apparently intact peroxisome biogenesis.		01.9910
Alcoholic Cirrhosis
[description not available]		01.9910
Liver Cirrhosis, Alcoholic
FIBROSIS of the hepatic parenchyma due to chronic excess ALCOHOL DRINKING.		01.9910
Convulsions, Grand Mal
[description not available]		02.420
Epilepsy, Tonic-Clonic
A generalized seizure disorder characterized by recurrent major motor seizures. The initial brief tonic phase is marked by trunk flexion followed by diffuse extension of the trunk and extremities. The clonic phase features rhythmic flexor contractions of the trunk and limbs, pupillary dilation, elevations of blood pressure and pulse, urinary incontinence, and tongue biting. This is followed by a profound state of depressed consciousness (post-ictal state) which gradually improves over minutes to hours. The disorder may be cryptogenic, familial, or symptomatic (caused by an identified disease process). (From Adams et al., Principles of Neurology, 6th ed, p329)		02.420
Bile Duct Obstruction
[description not available]		01.9910
Paralysis, Legs
[description not available]		01.9910
Cholestasis
Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).		01.9910
Paraplegia
Severe or complete loss of motor function in the lower extremities and lower portions of the trunk. This condition is most often associated with SPINAL CORD DISEASES, although BRAIN DISEASES; PERIPHERAL NERVOUS SYSTEM DISEASES; NEUROMUSCULAR DISEASES; and MUSCULAR DISEASES may also cause bilateral leg weakness.		01.9910
Metabolic Skin Diseases
[description not available]		02.9110
Cheilitis
Inflammation of the lips. It is of various etiologies and degrees of pathology.		02.9110
Apnea, Sleep
[description not available]		01.9910
Sleep Apnea Syndromes
Disorders characterized by multiple cessations of respirations during sleep that induce partial arousals and interfere with the maintenance of sleep. Sleep apnea syndromes are divided into central (see SLEEP APNEA, CENTRAL), obstructive (see SLEEP APNEA, OBSTRUCTIVE), and mixed central-obstructive types.		01.9910
Exanthem
[description not available]		01.9910
Exanthema
Diseases in which skin eruptions or rashes are a prominent manifestation. Classically, six such diseases were described with similar rashes; they were numbered in the order in which they were reported. Only the fourth (Duke's disease), fifth (ERYTHEMA INFECTIOSUM), and sixth (EXANTHEMA SUBITUM) numeric designations survive as occasional synonyms in current terminology.		01.9910
Acidosis, Renal Tubular, Type I
[description not available]		03.9850
Acidosis, Renal Tubular
A group of genetic disorders of the KIDNEY TUBULES characterized by the accumulation of metabolically produced acids with elevated plasma chloride, hyperchloremic metabolic ACIDOSIS. Defective renal acidification of URINE (proximal tubules) or low renal acid excretion (distal tubules) can lead to complications such as HYPOKALEMIA, hypercalcinuria with NEPHROLITHIASIS and NEPHROCALCINOSIS, and RICKETS.		03.9850
Palmoplantaris Pustulosis
[description not available]		01.9910
Psoriasis
A common genetically determined, chronic, inflammatory skin disease characterized by rounded erythematous, dry, scaling patches. The lesions have a predilection for nails, scalp, genitalia, extensor surfaces, and the lumbosacral region. Accelerated epidermopoiesis is considered to be the fundamental pathologic feature in psoriasis.		01.9910
Chronic Hepatitis
[description not available]		03.7821
Hepatitis, Chronic
INFLAMMATION of the LIVER with ongoing hepatocellular injury for 6 months or more, characterized by NECROSIS of HEPATOCYTES and inflammatory cell (LEUKOCYTES) infiltration. Chronic hepatitis can be caused by viruses, medications, autoimmune diseases, and other unknown factors.		03.7821
Albuminuria
The presence of albumin in the urine, an indicator of KIDNEY DISEASES.		03.3811
Adverse Drug Event
[description not available]		0210
Bone Marrow Diseases
Diseases involving the BONE MARROW.		0210
Alcoholic Liver Diseases
[description not available]		0210
Liver Diseases, Alcoholic
Liver diseases associated with ALCOHOLISM. It usually refers to the coexistence of two or more subentities, i.e., ALCOHOLIC FATTY LIVER; ALCOHOLIC HEPATITIS; and ALCOHOLIC CIRRHOSIS.		0210
Drug-Related Side Effects and Adverse Reactions
Disorders that result from the intended use of PHARMACEUTICAL PREPARATIONS. Included in this heading are a broad variety of chemically-induced adverse conditions due to toxicity, DRUG INTERACTIONS, and metabolic effects of pharmaceuticals.		0210
Critical Illness
A disease or state in which death is possible or imminent.		0210
Acute Onset Vascular Dementia
[description not available]		0210
Dementia, Vascular
An imprecise term referring to dementia associated with CEREBROVASCULAR DISORDERS, including CEREBRAL INFARCTION (single or multiple), and conditions associated with chronic BRAIN ISCHEMIA. Diffuse, cortical, and subcortical subtypes have been described. (From Gerontol Geriatr 1998 Feb;31(1):36-44)		0210
Optic Atrophy
Atrophy of the optic disk which may be congenital or acquired. This condition indicates a deficiency in the number of nerve fibers which arise in the RETINA and converge to form the OPTIC DISK; OPTIC NERVE; OPTIC CHIASM; and optic tracts. GLAUCOMA; ISCHEMIA; inflammation, a chronic elevation of intracranial pressure, toxins, optic nerve compression, and inherited conditions (see OPTIC ATROPHIES, HEREDITARY) are relatively common causes of this condition.		0210
Urinary Incontinence
Involuntary loss of URINE, such as leaking of urine. It is a symptom of various underlying pathological processes. Major types of incontinence include URINARY URGE INCONTINENCE and URINARY STRESS INCONTINENCE.		0210
Diseases of Immune System
[description not available]		02.9210
Immune System Diseases
Disorders caused by abnormal or absent immunologic mechanisms, whether humoral, cell-mediated, or both.		02.9210
Neutropenia
A decrease in the number of NEUTROPHILS found in the blood.		02.9210
Brain Swelling
[description not available]		0210
Astrocytosis
[description not available]		0210
Brain Edema
Increased intracellular or extracellular fluid in brain tissue. Cytotoxic brain edema (swelling due to increased intracellular fluid) is indicative of a disturbance in cell metabolism, and is commonly associated with hypoxic or ischemic injuries (see HYPOXIA, BRAIN). An increase in extracellular fluid may be caused by increased brain capillary permeability (vasogenic edema), an osmotic gradient, local blockages in interstitial fluid pathways, or by obstruction of CSF flow (e.g., obstructive HYDROCEPHALUS). (From Childs Nerv Syst 1992 Sep; 8(6):301-6)		0210
Anterior Choroidal Artery Infarction
[description not available]		02.9210
Cerebral Infarction
The formation of an area of NECROSIS in the CEREBRUM caused by an insufficiency of arterial or venous blood flow. Infarcts of the cerebrum are generally classified by hemisphere (i.e., left vs. right), lobe (e.g., frontal lobe infarction), arterial distribution (e.g., INFARCTION, ANTERIOR CEREBRAL ARTERY), and etiology (e.g., embolic infarction).		02.9210
Akinetic-Rigid Variant of Huntington Disease
[description not available]		0210
Huntington Disease
A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)		0210
Intraventricular Septal Defects
[description not available]		02.4120
Heart Septal Defects, Ventricular
Developmental abnormalities in any portion of the VENTRICULAR SEPTUM resulting in abnormal communications between the two lower chambers of the heart. Classification of ventricular septal defects is based on location of the communication, such as perimembranous, inlet, outlet (infundibular), central muscular, marginal muscular, or apical muscular defect.		02.4120
Esophageal Reflux
[description not available]		02.0110
Gastroesophageal Reflux
Retrograde flow of gastric juice (GASTRIC ACID) and/or duodenal contents (BILE ACIDS; PANCREATIC JUICE) into the distal ESOPHAGUS, commonly due to incompetence of the LOWER ESOPHAGEAL SPHINCTER.		02.0110
Deficiency, Vitamin E
[description not available]		02.3620
Alcaptonuria
[description not available]		03.5530
Carbohydrate Metabolism, Inborn Error
[description not available]		03.2820
ALDOB Deficiency
[description not available]		02.8610
Classic Galactosemia
[description not available]		04.4450
Glycogenosis
[description not available]		03.2720
Diabetes, Phosphate
[description not available]		02.8610
Adult Refsum Disease
[description not available]		02.8610
Alkaptonuria
An inborn error of amino acid metabolism resulting from a defect in the enzyme HOMOGENTISATE 1,2-DIOXYGENASE, an enzyme involved in the breakdown of PHENYLALANINE and TYROSINE. It is characterized by accumulation of HOMOGENTISIC ACID in the urine, OCHRONOSIS in various tissues, and ARTHRITIS.		03.5530
Galactosemias
A group of inherited enzyme deficiencies which feature elevations of GALACTOSE in the blood. This condition may be associated with deficiencies of GALACTOKINASE; UDPGLUCOSE-HEXOSE-1-PHOSPHATE URIDYLYLTRANSFERASE; or UDPGLUCOSE 4-EPIMERASE. The classic form is caused by UDPglucose-Hexose-1-Phosphate Uridylyltransferase deficiency, and presents in infancy with FAILURE TO THRIVE; VOMITING; and INTRACRANIAL HYPERTENSION. Affected individuals also may develop MENTAL RETARDATION; JAUNDICE; hepatosplenomegaly; ovarian failure (PRIMARY OVARIAN INSUFFICIENCY); and cataracts. (From Menkes, Textbook of Child Neurology, 5th ed, pp61-3)		04.4450
Glycogen Storage Disease
A group of inherited metabolic disorders involving the enzymes responsible for the synthesis and degradation of glycogen. In some patients, prominent liver involvement is presented. In others, more generalized storage of glycogen occurs, sometimes with prominent cardiac involvement.		03.2720
Hypophosphatemia, Familial
An inherited condition of abnormally low serum levels of PHOSPHATES (below 1 mg/liter) which can occur in a number of genetic diseases with defective reabsorption of inorganic phosphorus by the PROXIMAL RENAL TUBULES. This leads to phosphaturia, HYPOPHOSPHATEMIA, and disturbances of cellular and organ functions such as those in X-LINKED HYPOPHOSPHATEMIC RICKETS; OSTEOMALACIA; and FANCONI SYNDROME.		02.8610
Emergencies
Situations or conditions requiring immediate intervention to avoid serious adverse results.		01.9510
Deficiency, Glucosephosphatase
[description not available]		03.7420
Deficiency, Hexosediphosphatase
[description not available]		02.8710
Acute Hemorrhagic Encephalomyelitis
[description not available]		02.8710
Glycogen Storage Disease Type I
An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood.		03.7420
Aminoaciduria, Renal
[description not available]		02.8710
Kahler Disease
[description not available]		01.9510
Multiple Myeloma
A malignancy of mature PLASMA CELLS engaging in monoclonal immunoglobulin production. It is characterized by hyperglobulinemia, excess Bence-Jones proteins (free monoclonal IMMUNOGLOBULIN LIGHT CHAINS) in the urine, skeletal destruction, bone pain, and fractures. Other features include ANEMIA; HYPERCALCEMIA; and RENAL INSUFFICIENCY.		01.9510
Besnoitiasis
[description not available]		01.9510
Poultry Diseases
Diseases of birds which are raised as a source of meat or eggs for human consumption and are usually found in barnyards, hatcheries, etc. The concept is differentiated from BIRD DISEASES which is for diseases of birds not considered poultry and usually found in zoos, parks, and the wild.		01.9510
Alactasia
[description not available]		02.8710
Lactose Intolerance
The condition resulting from the absence or deficiency of LACTASE in the MUCOSA cells of the GASTROINTESTINAL TRACT, and the inability to break down LACTOSE in milk for ABSORPTION. Bacterial fermentation of the unabsorbed lactose leads to symptoms that range from a mild indigestion (DYSPEPSIA) to severe DIARRHEA. Lactose intolerance may be an inborn error or acquired.		02.8710
Cot Death
[description not available]		02.3720
Ataxia with Lactic Acidosis
[description not available]		01.9510
Pyruvate Dehydrogenase Complex Deficiency Disease
An inherited metabolic disorder caused by deficient enzyme activity in the PYRUVATE DEHYDROGENASE COMPLEX, resulting in deficiency of acetyl CoA and reduced synthesis of acetylcholine. Two clinical forms are recognized: neonatal and juvenile. The neonatal form is a relatively common cause of lactic acidosis in the first weeks of life and may also feature an erythematous rash. The juvenile form presents with lactic acidosis, alopecia, intermittent ATAXIA; SEIZURES; and an erythematous rash. (From J Inherit Metab Dis 1996;19(4):452-62) Autosomal recessive and X-linked forms are caused by mutations in the genes for the three different enzyme components of this multisubunit pyruvate dehydrogenase complex. One of the mutations at Xp22.2-p22.1 in the gene for the E1 alpha component of the complex leads to LEIGH DISEASE.		01.9510
Choreoathetosis Self-Mutilation Hyperuricemia Syndrome
[description not available]		01.9510
Lesch-Nyhan Syndrome
An inherited disorder transmitted as a sex-linked trait and caused by a deficiency of an enzyme of purine metabolism; HYPOXANTHINE PHOSPHORIBOSYLTRANSFERASE. Affected individuals are normal in the first year of life and then develop psychomotor retardation, extrapyramidal movement disorders, progressive spasticity, and seizures. Self-destructive behaviors such as biting of fingers and lips are seen frequently. Intellectual impairment may also occur but is typically not severe. Elevation of uric acid in the serum leads to the development of renal calculi and gouty arthritis. (Menkes, Textbook of Child Neurology, 5th ed, pp127)		01.9510
Liver Steatosis
[description not available]		01.9510
Fatty Liver with Encephalopathy
[description not available]		02.3620
Fatty Liver
Lipid infiltration of the hepatic parenchymal cells resulting in a yellow-colored liver. The abnormal lipid accumulation is usually in the form of TRIGLYCERIDES, either as a single large droplet or multiple small droplets. Fatty liver is caused by an imbalance in the metabolism of FATTY ACIDS.		01.9510
Poisoning
Used with drugs, chemicals, and industrial materials for human or animal poisoning, acute or chronic, whether the poisoning is accidental, occupational, suicidal, by medication error, or by environmental exposure.		01.9810
Eye Disorders
[description not available]		01.9810
Iris Diseases
Diseases, dysfunctions, or disorders of or located in the iris.		01.9810
Eye Diseases
Diseases affecting the eye.		01.9810
Blood Poisoning
[description not available]		01.9810
Sepsis
Systemic inflammatory response syndrome with a proven or suspected infectious etiology. When sepsis is associated with organ dysfunction distant from the site of infection, it is called severe sepsis. When sepsis is accompanied by HYPOTENSION despite adequate fluid infusion, it is called SEPTIC SHOCK.		01.9810
Cirrhosis, Liver
[description not available]		01.9810
Hepatitis
INFLAMMATION of the LIVER.		01.9810
Liver Cirrhosis
Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.		01.9810
Congenital Adrenal Hyperplasia
[description not available]		03.5830
Adrenal Hyperplasia, Congenital
A group of inherited disorders of the ADRENAL GLANDS, caused by enzyme defects in the synthesis of cortisol (HYDROCORTISONE) and/or ALDOSTERONE leading to accumulation of precursors for ANDROGENS. Depending on the hormone imbalance, congenital adrenal hyperplasia can be classified as salt-wasting, hypertensive, virilizing, or feminizing. Defects in STEROID 21-HYDROXYLASE; STEROID 11-BETA-HYDROXYLASE; STEROID 17-ALPHA-HYDROXYLASE; 3-beta-hydroxysteroid dehydrogenase (3-HYDROXYSTEROID DEHYDROGENASES); TESTOSTERONE 5-ALPHA-REDUCTASE; or steroidogenic acute regulatory protein; among others, underlie these disorders.		03.5830
Thyrotoxicosis
A hypermetabolic syndrome caused by excess THYROID HORMONES which may come from endogenous or exogenous sources. The endogenous source of hormone may be thyroid HYPERPLASIA; THYROID NEOPLASMS; or hormone-producing extrathyroidal tissue. Thyrotoxicosis is characterized by NERVOUSNESS; TACHYCARDIA; FATIGUE; WEIGHT LOSS; heat intolerance; and excessive SWEATING.		02.8910
Fetal Death
Death of the developing young in utero. BIRTH of a dead FETUS is STILLBIRTH.		01.9710
Acquired Immune Deficiency Syndrome
[description not available]		01.9710
Acquired Immunodeficiency Syndrome
An acquired defect of cellular immunity associated with infection by the human immunodeficiency virus (HIV), a CD4-positive T-lymphocyte count under 200 cells/microliter or less than 14% of total lymphocytes, and increased susceptibility to opportunistic infections and malignant neoplasms. Clinical manifestations also include emaciation (wasting) and dementia. These elements reflect criteria for AIDS as defined by the CDC in 1993.		01.9710
Mucorales Infection
[description not available]		01.9710
Mucormycosis
Infection in humans and animals caused by any fungus in the order MUCORALES (e.g., RHIZOPUS; MUCOR; CUNNINGHAMELLA; APOPHYSOMYCES; ABSIDIA; SAKSENAEA and RHIZOMUCOR) There are many clinical types associated with infection including central nervous system, lung, gastrointestinal tract, skin, orbit and paranasal sinuses. In humans, it usually occurs as an OPPORTUNISTIC INFECTION.		01.9710
Adipocere
[description not available]		01.9710
Embolism, Pulmonary
[description not available]		01.9710
Cor Pulmonale
[description not available]		06.9710
Pulmonary Embolism
Blocking of the PULMONARY ARTERY or one of its branches by an EMBOLUS.		01.9710
Appetite Disorders
[description not available]		01.9710
Child Behavior Disorders
Disturbances considered to be pathological based on age and stage appropriateness, e.g., conduct disturbances and anaclitic depression. This concept does not include psychoneuroses, psychoses, or personality disorders with fixed patterns.		01.9710
Feeding and Eating Disorders
A group of disorders characterized by physiological and psychological disturbances in appetite or food intake.		01.9710
Aldosteronism
[description not available]		01.9710
Hyperpotassemia
[description not available]		01.9710
Hyperaldosteronism
A condition caused by the overproduction of ALDOSTERONE. It is characterized by sodium retention and potassium excretion with resultant HYPERTENSION and HYPOKALEMIA.		01.9710
Hyperkalemia
Abnormally high potassium concentration in the blood, most often due to defective renal excretion. It is characterized clinically by electrocardiographic abnormalities (elevated T waves and depressed P waves, and eventually by atrial asystole). In severe cases, weakness and flaccid paralysis may occur. (Dorland, 27th ed)		01.9710
Alloxan Diabetes
[description not available]		01.9710
Academic Disorder, Developmental
[description not available]		01.9710
Auditory Processing Disorder, Central
[description not available]		01.9710
Learning Disabilities
Conditions characterized by a significant discrepancy between an individual's perceived level of intellect and their ability to acquire new language and other cognitive skills. These may result from organic or psychological conditions. Relatively common subtypes include DYSLEXIA, DYSCALCULIA, and DYSGRAPHIA.		01.9710
Antibiotic-Associated Colitis
[description not available]		01.9710
Enterocolitis, Pseudomembranous
An acute inflammation of the INTESTINAL MUCOSA that is characterized by the presence of pseudomembranes or plaques in the SMALL INTESTINE (pseudomembranous enteritis) and the LARGE INTESTINE (pseudomembranous colitis). It is commonly associated with antibiotic therapy and CLOSTRIDIUM DIFFICILE colonization.		01.9710
Brain Damage, Chronic
A condition characterized by long-standing brain dysfunction or damage, usually of three months duration or longer. Potential etiologies include BRAIN INFARCTION; certain NEURODEGENERATIVE DISORDERS; CRANIOCEREBRAL TRAUMA; ANOXIA, BRAIN; ENCEPHALITIS; certain NEUROTOXICITY SYNDROMES; metabolic disorders (see BRAIN DISEASES, METABOLIC); and other conditions.		01.9610
Aganglionic Megacolon
[description not available]		01.9710
Hirschsprung Disease
Congenital MEGACOLON resulting from the absence of ganglion cells (aganglionosis) in a distal segment of the LARGE INTESTINE. The aganglionic segment is permanently contracted thus causing dilatation proximal to it. In most cases, the aganglionic segment is within the RECTUM and SIGMOID COLON.		01.9710
Brain Vascular Disorders
[description not available]		01.9710
Cerebrovascular Disorders
A spectrum of pathological conditions of impaired blood flow in the brain. They can involve vessels (ARTERIES or VEINS) in the CEREBRUM, the CEREBELLUM, and the BRAIN STEM. Major categories include INTRACRANIAL ARTERIOVENOUS MALFORMATIONS; BRAIN ISCHEMIA; CEREBRAL HEMORRHAGE; and others.		01.9710
Central Hypothyroidism
[description not available]		01.9710
Hypothyroidism
A syndrome that results from abnormally low secretion of THYROID HORMONES from the THYROID GLAND, leading to a decrease in BASAL METABOLIC RATE. In its most severe form, there is accumulation of MUCOPOLYSACCHARIDES in the SKIN and EDEMA, known as MYXEDEMA. It may be primary or secondary due to other pituitary disease, or hypothalamic dysfunction.		01.9710
Hypesthesia
Absent or reduced sensitivity to cutaneous stimulation.		01.9710
Arrhythmia
[description not available]		01.9610
Arrhythmias, Cardiac
Any disturbances of the normal rhythmic beating of the heart or MYOCARDIAL CONTRACTION. Cardiac arrhythmias can be classified by the abnormalities in HEART RATE, disorders of electrical impulse generation, or impulse conduction.		01.9610
Bladder Cancer
[description not available]		01.9610
Cancer of Ovary
[description not available]		01.9610
Urinary Bladder Neoplasms
Tumors or cancer of the URINARY BLADDER.		01.9610
Ovarian Neoplasms
Tumors or cancer of the OVARY. These neoplasms can be benign or malignant. They are classified according to the tissue of origin, such as the surface EPITHELIUM, the stromal endocrine cells, and the totipotent GERM CELLS.		01.9610
Leucocythaemia
[description not available]		01.9510
Gout
Metabolic disorder characterized by recurrent acute arthritis, hyperuricemia and deposition of sodium urate in and around the joints, sometimes with formation of URIC ACID calculi.		01.9510
Leukemia
A progressive, malignant disease of the blood-forming organs, characterized by distorted proliferation and development of leukocytes and their precursors in the blood and bone marrow. Leukemias were originally termed acute or chronic based on life expectancy but now are classified according to cellular maturity. Acute leukemias consist of predominately immature cells; chronic leukemias are composed of more mature cells. (From The Merck Manual, 2006)		01.9510
Deficiency of Glucose-6-Phosphate Dehydrogenase
[description not available]		02.8610
Glucosephosphate Dehydrogenase Deficiency
A disease-producing enzyme deficiency subject to many variants, some of which cause a deficiency of GLUCOSE-6-PHOSPHATE DEHYDROGENASE activity in erythrocytes, leading to hemolytic anemia.		02.8610
Cystine Diathesis
[description not available]		02.3520
Albinism
General term for a number of inherited defects of amino acid metabolism in which there is a deficiency or absence of pigment in the eyes, skin, or hair.		02.3520
Cystinosis
A metabolic disease characterized by the defective transport of CYSTINE across the lysosomal membrane due to mutation of a membrane protein cystinosin. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. In the KIDNEY, nephropathic cystinosis is a common cause of RENAL FANCONI SYNDROME.		02.3520