phenylacetylglutamine profile

N(2)-phenylacetyl-L-glutamine : An a N(2)-phenylacetylglutamine having L-configuration. [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]

ID Source	ID
PubMed CID	92258
CHEMBL ID	4303372
CHEBI ID	17884
SCHEMBL ID	236527
MeSH ID	M0044036

Synonym
l-glutamine, n2-(phenylacetyl)-
phenylacetyl-l-glutamine
n(2)-phenylacetyl-l-glutamine
n(2)-(phenylacetyl)-l-glutamine
CHEBI:17884
glutamine, n2-(phenylacetyl)-, l-
pa-l-glutamine
brn 2593680
l-n(sup 2)-(phenylacetyl)glutamine
nsc 203800
glutamine, n(sup 2)-(phenylacetyl)-, l-
phenylacetylglutamine
C04148
28047-15-6
n2-(2-phenylacetyl)-l-glutamine
alpha-n-phenylacetyl-l-glutamine
(s)-5-amino-5-oxo-2-(2-phenylacetamido)pentanoic acid
A819301
3-09-00-02227 (beilstein handbook reference)
92358i79rg ,
unii-92358i79rg
AKOS010385089
phenylacetyl l-glutamine
SCHEMBL236527
FD21545
phenylac-gln-oh
DTXSID90182324
mfcd01725188
l-n(sup 2)-(phenylacetyl)
(2s)-4-carbamoyl-2-(2-phenylacetamido)butanoic acid
phenylac-gln-oh, aldrichcpr
n-phenylacetyl-l-glutamine (phenylacetylglutamine)
(s)-5-amino-5-oxo-2-(2-phenylacetamido)pentanoicacid
phenylacetylglutamine [mi]
astugenal component phenylacetylglutamine
antineoplaston as2-1 component phenylacetylglutamine
antineoplaston as 2-1 component phenylacetylglutamine
phenylacetylglutamine [who-dd]
HY-W050026
CS-0031369
DS-16807
Q7181415
phenyl-ac-gln-oh
CHEMBL4303372
S6076

Excerpt	Reference	Relevance
"Phenylacetylglutamine (PAG) is a metabolite that is excreted in human urine. "	( Urinary phenylacetylglutamine as a possible biomarker for central nervous system disorders in forensic autopsy cases. Hara, K; Ikematsu, N; Kashiwagi, M; Kubo, SI; Matsusue, A; Takayama, M; Waters, B, 2023)	2.79

Excerpt	Reference	Relevance
" Adverse events were comparable for the two drugs; 2 subjects experienced hyperammonemic events on NaPBA while none occurred on GPB."	( Phase 2 comparison of a novel ammonia scavenging agent with sodium phenylbutyrate in patients with urea cycle disorders: safety, pharmacokinetics and ammonia control. Beliveau, M; Berry, SA; Diaz, GA; Dickinson, K; Gargosky, S; Lee, B; Marier, JF; Martinez, A; Mauney, J; Mian, A; Mokhtarani, M; Rhead, W; Scharschmidt, BF; Shchelochkov, O, 2010)	0.36
" OP is safe in healthy subjects and in stable patients with cirrhosis, but there are no data in decompensated cirrhosis."	( Safety of ornithine phenylacetate in cirrhotic decompensated patients: an open-label, dose-escalating, single-cohort study. Arranz, JA; Blanco, A; Córdoba, J; Fuentes, I; Riudor, E; Simón-Talero, M; Soriano, G; Suñé, P; Torrens, M; Ventura-Cots, M, )	0.13
"No severe adverse events were observed."	( Safety of ornithine phenylacetate in cirrhotic decompensated patients: an open-label, dose-escalating, single-cohort study. Arranz, JA; Blanco, A; Córdoba, J; Fuentes, I; Riudor, E; Simón-Talero, M; Soriano, G; Suñé, P; Torrens, M; Ventura-Cots, M, )	0.13
"OP is a safe and well-tolerated drug in decompensated cirrhotics that may decrease plasma ammonia by inducing its appearance as phenylacetylglutamine in urine."	( Safety of ornithine phenylacetate in cirrhotic decompensated patients: an open-label, dose-escalating, single-cohort study. Arranz, JA; Blanco, A; Córdoba, J; Fuentes, I; Riudor, E; Simón-Talero, M; Soriano, G; Suñé, P; Torrens, M; Ventura-Cots, M, )	0.34
" PAA plasma levels ≥ 500 μg/dL have been reported to be associated with reversible neurological adverse events (AEs) in cancer patients receiving PAA intravenously."	( Elevated phenylacetic acid levels do not correlate with adverse events in patients with urea cycle disorders or hepatic encephalopathy and can be predicted based on the plasma PAA to PAGN ratio. Bartley, J; Berquist, W; Berry, SA; Brown, RS; Coakley, D; Diaz, GA; Dickinson, K; Feigenbaum, A; Gallagher, R; Ghabril, M; Harding, C; Lee, B; Lemons, C; Lichter-Konecki, U; Longo, N; Mantry, P; McCandless, SE; Milikien, DA; Mokhtarani, M; Moors, T; Nagamani, SC; Norris, C; Rhead, W; Rockey, DC; Scharschmidt, BF; Schulze, A; Smith, W; Vierling, JM, 2013)	0.39
" Of the reported serious adverse events (AEs), which included 11 deaths, none was attributable to study medication."	( Safety, tolerability, and pharmacokinetics of l-ornithine phenylacetate in patients with acute liver injury/failure and hyperammonemia. Bukofzer, S; Clasen, K; Durkalski, V; Fontana, RJ; Ganger, D; Gottfried, M; Hameed, B; Hanje, AJ; Koch, D; Lee, WM; Little, L; Ravis, WR; Sherker, A; Stravitz, RT; Subramanian, RM, 2018)	0.48
" Phenylacetic acid (PAA) plasma exposure has been reported to correlate with neurologic adverse events in patients with cancer but not in patients with urea cycle disorders or hepatic encephalopathy."	( Exposures of Phenylacetic Acid and Phenylacetylglutamine Across Different Subpopulations and Correlation with Adverse Events. Mak, C; Poola, N; Tseng, J; Vilchez, RA; Wang, X, 2021)	0.9
"This analysis summarized the pharmacokinetics and safety of ornithine phenylacetate to support the dosing strategy and to assist with the monitoring and management of neurologic adverse events in a global clinical development program."	( Exposures of Phenylacetic Acid and Phenylacetylglutamine Across Different Subpopulations and Correlation with Adverse Events. Mak, C; Poola, N; Tseng, J; Vilchez, RA; Wang, X, 2021)	0.9
"Phenylacetic acid and phenylacetylglutamine (PAGN) pharmacokinetic data and adverse events from five clinical studies were included in the analysis."	( Exposures of Phenylacetic Acid and Phenylacetylglutamine Across Different Subpopulations and Correlation with Adverse Events. Mak, C; Poola, N; Tseng, J; Vilchez, RA; Wang, X, 2021)	1.21
" No correlation was observed between PAA plasma exposure and neurologic adverse events in patients with stable cirrhosis or acute hepatic encephalopathy."	( Exposures of Phenylacetic Acid and Phenylacetylglutamine Across Different Subpopulations and Correlation with Adverse Events. Mak, C; Poola, N; Tseng, J; Vilchez, RA; Wang, X, 2021)	0.9
" Phenylacetic acid plasma exposure was not correlated with neurologic adverse events in the ornithine phenylacetate target patient population."	( Exposures of Phenylacetic Acid and Phenylacetylglutamine Across Different Subpopulations and Correlation with Adverse Events. Mak, C; Poola, N; Tseng, J; Vilchez, RA; Wang, X, 2021)	0.9

Excerpt	Reference	Relevance
" We performed a pharmacokinetic study, as part of a phase I trial, of PAA in children with refractory cancer."	( Pharmacokinetics of phenylacetate administered as a 30-min infusion in children with refractory cancer. Adamson, P; Aiken, A; Balis, F; Berg, S; Blaney, SM; Jakacki, R; Klenke, R; Murry, DJ; Packer, R; Serabe, BM; Thompson, P, 2003)	0.32
"The capacity-limited conversion of PAA to PAG has important implications for the dosing of PAA, and the pharmacokinetic model described here may be useful for individualizing the infusion rate of the drug in future clinical trials."	( Pharmacokinetics of phenylacetate administered as a 30-min infusion in children with refractory cancer. Adamson, P; Aiken, A; Balis, F; Berg, S; Blaney, SM; Jakacki, R; Klenke, R; Murry, DJ; Packer, R; Serabe, BM; Thompson, P, 2003)	0.32

Excerpt	Reference	Relevance
" The antitumour effects of interferon alpha (IFN alpha) in combination with AS2-1, the hydrolysis product of 3-phenylacetyl-amino-2,6-piperidinedione, were examined using several human tumour cell lines as a model."	( Interferon in combination with antitumourigenic phenyl derivatives: potentiation of IFN alpha activity in-vitro. Samid, D; Shack, S; Yeh, TJ, 1991)	0.28

Excerpt	Reference	Relevance
" bioavailability determination."	( A phase I dose escalation and bioavailability study of oral sodium phenylbutyrate in patients with refractory solid tumor malignancies. Baker, SD; Bowling, MK; Carducci, MA; Donehower, RC; Figg, WD; Gilbert, J; Grochow, L; Zabelina, Y, 2001)	0.31

Excerpt	Relevance	Reference
" Combined UV, 1H NMR, and positive-ion electrospray MS detection was achieved in the continuous-flow mode using whole human urine from a subject dosed with acetaminophen."	( Combined HPLC, NMR spectroscopy, and ion-trap mass spectrometry with application to the detection and characterization of xenobiotic and endogenous metabolites in human urine. Foxall, PJ; Lindon, JC; Nicholson, JK; Shockcor, JP; Unger, SE; Wilson, ID, 1996)	0.29
"The capacity-limited conversion of PAA to PAG has important implications for the dosing of PAA, and the pharmacokinetic model described here may be useful for individualizing the infusion rate of the drug in future clinical trials."	( Pharmacokinetics of phenylacetate administered as a 30-min infusion in children with refractory cancer. Adamson, P; Aiken, A; Balis, F; Berg, S; Blaney, SM; Jakacki, R; Klenke, R; Murry, DJ; Packer, R; Serabe, BM; Thompson, P, 2003)	0.32
" Blood ammonia and blood and urine metabolites were compared after 7 days (steady state) of TID dosing on either drug, both dosed to deliver the same amount of phenylbutyric acid (PBA)."	( Phase 2 comparison of a novel ammonia scavenging agent with sodium phenylbutyrate in patients with urea cycle disorders: safety, pharmacokinetics and ammonia control. Beliveau, M; Berry, SA; Diaz, GA; Dickinson, K; Gargosky, S; Lee, B; Marier, JF; Martinez, A; Mauney, J; Mian, A; Mokhtarani, M; Rhead, W; Scharschmidt, BF; Shchelochkov, O, 2010)	0.36
" Twenty-four healthy adults underwent single-dose administration of GPB and NaPBA and eight healthy adults and 24 cirrhotic subjects underwent single-day and multiple-day dosing of GPB, with metabolites measured in blood and urine."	( Pharmacology and safety of glycerol phenylbutyrate in healthy adults and adults with cirrhosis. Dickinson, K; Gargosky, S; Lowe, ME; Martinez, A; McGuire, BM; Mokhtarani, M; Monteleone, J; Scharschmidt, BF; Syplyviy, VA; Xiao, X; Zupanets, IA, 2010)	0.36
"We have analyzed pharmacokinetic data for glycerol phenylbutyrate (also GT4P or HPN-100) and sodium phenylbutyrate with respect to possible dosing biomarkers in patients with urea cycle disorders (UCD)."	( Urinary phenylacetylglutamine as dosing biomarker for patients with urea cycle disorders. Bart, S; Bartholomew, D; Bartley, J; Berquist, W; Berry, SA; Cederbaum, S; Coakley, DF; Diaz, GA; Dickinson, K; Dorrani, N; Feigenbaum, A; Gallagher, R; Harding, CO; Korson, MS; Kronn, D; Lee, B; Lemons, C; Lichter-Konecki, U; Longo, N; McCandless, SE; Merritt, JL; Mokhtarani, M; Moors, TL; Rhead, W; Scharschmidt, BF; Smith, W; Sreenath-Nagamani, S; Summar, M; Vockley, J; Zori, R, 2012)	0.81
" Dose simulations demonstrated similar PAA exposure following mole-equivalent PBA dosing of both drugs and greater PAA exposure in younger patients based on BSA."	( Population pharmacokinetic modeling and dosing simulations of nitrogen-scavenging compounds: disposition of glycerol phenylbutyrate and sodium phenylbutyrate in adult and pediatric patients with urea cycle disorders. Berry, SA; Coakley, D; Diaz, GA; Dickinson, K; Lee, B; Lemons, C; Lichter-Konecki, U; Mokhtarani, M; Monteleone, JP; Rhead, W; Scharschmidt, BF, 2013)	0.39
" The plasma PAA:PAGN ratio is a functional measure of the rate of PAA metabolism and represents a useful dosing biomarker."	( Elevated phenylacetic acid levels do not correlate with adverse events in patients with urea cycle disorders or hepatic encephalopathy and can be predicted based on the plasma PAA to PAGN ratio. Bartley, J; Berquist, W; Berry, SA; Brown, RS; Coakley, D; Diaz, GA; Dickinson, K; Feigenbaum, A; Gallagher, R; Ghabril, M; Harding, C; Lee, B; Lemons, C; Lichter-Konecki, U; Longo, N; Mantry, P; McCandless, SE; Milikien, DA; Mokhtarani, M; Moors, T; Nagamani, SC; Norris, C; Rhead, W; Rockey, DC; Scharschmidt, BF; Schulze, A; Smith, W; Vierling, JM, 2013)	0.39
" Ornithine phenylacetate, an intravenous dosage form of the L-ornithine salt of phenylacetate, is under development for hepatic encephalopathy."	( Exposures of Phenylacetic Acid and Phenylacetylglutamine Across Different Subpopulations and Correlation with Adverse Events. Mak, C; Poola, N; Tseng, J; Vilchez, RA; Wang, X, 2021)	0.9
"This analysis summarized the pharmacokinetics and safety of ornithine phenylacetate to support the dosing strategy and to assist with the monitoring and management of neurologic adverse events in a global clinical development program."	( Exposures of Phenylacetic Acid and Phenylacetylglutamine Across Different Subpopulations and Correlation with Adverse Events. Mak, C; Poola, N; Tseng, J; Vilchez, RA; Wang, X, 2021)	0.9
" Elevated PAGln levels were independently associated with a higher risk of the primary endpoint in a dose-response manner, regardless of HF subtype."	( Increased circulating phenylacetylglutamine concentration elevates the predictive value of cardiovascular event risk in heart failure patients. Chen, C; Huang, J; Li, C; Peng, L; Song, Y; Sun, Y; Wang, DW; Wang, H; Wei, H; Wu, J; Xiao, L; Zhang, Y; Zhao, C; Zhou, Z, 2023)	1.22

Role	Description
human metabolite	Any 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]

Class	Description
N(2)-phenylacetylglutamine
[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]

Pathway	Proteins	Compounds
Phenylacetate Metabolism	3	8

Timeframe	Studies, This Drug (%)	All Drugs %
pre-1990	11 (13.58)	18.7374
1990's	14 (17.28)	18.2507
2000's	9 (11.11)	29.6817
2010's	26 (32.10)	24.3611
2020's	21 (25.93)	2.80
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Publication Type	This drug (%)	All Drugs (%)
Trials	14 (16.47%)	5.53%
Reviews	3 (3.53%)	6.00%
Case Studies	4 (4.71%)	4.05%
Observational	1 (1.18%)	0.25%
Other	63 (74.12%)	84.16%
[information is prepared from research data collected from National Library of Medicine (NLM), extracted Dec-2023]

Substance	Relationship Strength	Studies	Trials	Classes	Roles
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.	3.9	2	1	O-acylcarnitine	human metabolite
alpha-ketoisovalerate alpha-ketoisovalerate: RN given refers to parent cpd. 3-methyl-2-oxobutanoate : A 2-oxo monocarboxylic acid anion that is the conjugate base of 3-methyl-2-oxobutanoic acid, arising from deprotonation of the carboxy group.. 3-methyl-2-oxobutanoic acid : A 2-oxo monocarboxylic acid that is the 2-oxo derivative of isovaleric acid.	2.25	1	0	2-oxo monocarboxylic acid; branched-chain keto acid	human metabolite; Saccharomyces cerevisiae metabolite
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.	3.98	2	1	monocarboxylic acid; phenols	fungal metabolite; human metabolite; mouse metabolite; plant metabolite
ammonium hydroxide azane : Saturated acyclic nitrogen hydrides having the general formula NnHn+2.	8.38	12	5	azane; 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
benzoic acid Benzoic Acid: A fungistatic compound that is widely used as a food preservative. It is conjugated to GLYCINE in the liver and excreted as hippuric acid.. benzoic acid : A compound comprising a benzene ring core carrying a carboxylic acid substituent.. aromatic carboxylic acid : Any carboxylic acid in which the carboxy group is directly bonded to an aromatic ring.	1.96	1	0	benzoic acids	algal metabolite; antimicrobial food preservative; drug allergen; EC 1.13.11.33 (arachidonate 15-lipoxygenase) inhibitor; EC 3.1.1.3 (triacylglycerol lipase) inhibitor; human xenobiotic metabolite; plant metabolite
carnitine [no description available]	5.47	3	1	amino-acid betaine	human metabolite; mouse metabolite
choline [no description available]	3.35	1	0	cholines	allergen; 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.	2.05	1	0	tricarboxylic acid	antimicrobial agent; chelator; food acidity regulator; fundamental 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.	2.25	1	0	(omega-1)-hydroxy fatty acid; 3-hydroxy monocarboxylic acid; hydroxybutyric acid	human metabolite
hippuric acid hippuric acid: RN given refers to parent cpd; structure in Merck Index, 9th ed, #4591. N-benzoylglycine : An N-acylglycine in which the acyl group is specified as benzoyl.	10.73	7	1	N-acylglycine	human blood serum metabolite; uremic toxin
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.	1.99	1	0	2-hydroxy monocarboxylic acid	algal metabolite; Daphnia magna metabolite
glycerol Moon: The natural satellite of the planet Earth. It includes the lunar cycles or phases, the lunar month, lunar landscapes, geography, and soil.	9.48	13	5	alditol; triol	algal metabolite; detergent; Escherichia coli metabolite; geroprotector; human metabolite; mouse metabolite; osmolyte; Saccharomyces cerevisiae metabolite; solvent
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.	1.98	1	0	carbon oxoanion	cofactor; 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.	3.8	2	0	elemental hydrogen; elemental molecule; gas molecular entity	antioxidant; electron donor; food packaging gas; fuel; human metabolite
kynurenine Kynurenine: A metabolite of the essential amino acid tryptophan metabolized via the tryptophan-kynurenine pathway.. kynurenine : A ketone that is alanine in which one of the methyl hydrogens is substituted by a 2-aminobenzoyl group.	2.13	1	0	aromatic ketone; non-proteinogenic alpha-amino acid; substituted aniline	human metabolite
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.	1.96	1	0	pyrimidinemonocarboxylic acid	Escherichia coli metabolite; metabolite; mouse metabolite
phenylpyruvic acid phenylpyruvic acid: RN given refers to parent cpd. phenylpyruvate : A 2-oxo monocarboxylic acid anion resulting from deprotonation of the carboxy group of either keto- or enol-phenylpyruvic acid.. keto-phenylpyruvic acid : A 2-oxo monocarboxylic acid that is 3-phenylpropanoic acid substituted by an oxo group at position 2. It is an intermediate metabolite in the phenylalanine pathway.	2.6	1	0	2-oxo monocarboxylic acid	chromogenic compound; EC 6.4.1.1 (pyruvate carboxylase) inhibitor; fundamental metabolite
phenylacetic acid phenylacetic acid : A monocarboxylic acid that is toluene in which one of the hydrogens of the methyl group has been replaced by a carboxy group.	15.04	27	6	benzenes; monocarboxylic acid; phenylacetic acids	allergen; Aspergillus metabolite; auxin; EC 6.4.1.1 (pyruvate carboxylase) inhibitor; Escherichia coli metabolite; human metabolite; plant growth retardant; plant metabolite; Saccharomyces cerevisiae metabolite; toxin
phosphoenolpyruvate Phosphoenolpyruvate: A monocarboxylic acid anion derived from selective deprotonation of the carboxy group of phosphoenolpyruvic acid. It is a metabolic intermediate in GLYCOLYSIS; GLUCONEOGENESIS; and other pathways.. phosphoenolpyruvate : A monocarboxylic acid anion resuting from selective deprotonation of the carboxy group of phosphoenolpyruvic acid.. phosphoenolpyruvic acid : A monocarboxylic acid that is acrylic acid substituted by a phosphonooxy group at position 2. It is a metabolic intermediate in pathways like glycolysis and gluconeogenesis.	3.8	2	0	carboxyalkyl phosphate; monocarboxylic acid	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.	1.96	1	0	pyridinedicarboxylic acid	Escherichia coli metabolite; human metabolite; mouse metabolite; NMDA receptor agonist
trimethyloxamine trimethyloxamine: used in manufacture of quaternary ammonium cpds; insect attractant; warming agent for gas; oxidant; structure. trimethylamine N-oxide : A tertiary amine oxide resulting from the oxidation of the amino group of trimethylamine.	5.74	4	1	tertiary amine oxide	Escherichia coli metabolite; metabolite; osmolyte
trimethylamine [no description available]	3.64	1	1	methylamines; tertiary amine	Escherichia coli metabolite; human xenobiotic 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.	3.39	1	1	uric acid	Escherichia 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.	12.25	9	2	isourea; monocarboxylic acid amide; one-carbon compound	Daphnia magna metabolite; Escherichia coli metabolite; fertilizer; flour treatment agent; human metabolite; mouse metabolite; Saccharomyces cerevisiae metabolite
1-anilino-8-naphthalenesulfonate 1-anilino-8-naphthalenesulfonate: RN given refers to parent cpd. 8-anilinonaphthalene-1-sulfonic acid : A naphthalenesulfonic acid that is naphthalene-1-sulfonic acid substituted by a phenylamino group at position 8.	5.79	2	2	aminonaphthalene; naphthalenesulfonic acid	fluorescent probe
acetaminophen Acetaminophen: Analgesic antipyretic derivative of acetanilide. It has weak anti-inflammatory properties and is used as a common analgesic, but may cause liver, blood cell, and kidney damage.. paracetamol : A member of the class of phenols that is 4-aminophenol in which one of the hydrogens attached to the amino group has been replaced by an acetyl group.	4.48	5	0	acetamides; phenols	antipyretic; cyclooxygenase 1 inhibitor; cyclooxygenase 2 inhibitor; cyclooxygenase 3 inhibitor; environmental contaminant; ferroptosis inducer; geroprotector; hepatotoxic agent; human blood serum metabolite; non-narcotic analgesic; non-steroidal anti-inflammatory drug; xenobiotic
amitriptyline Amitriptyline: Tricyclic antidepressant with anticholinergic and sedative properties. It appears to prevent the re-uptake of norepinephrine and serotonin at nerve terminals, thus potentiating the action of these neurotransmitters. Amitriptyline also appears to antagonize cholinergic and alpha-1 adrenergic responses to bioactive amines.. amitriptyline : An organic tricyclic compound that is 10,11-dihydro-5H-dibenzo[a,d][7]annulene substituted by a 3-(dimethylamino)propylidene group at position 5.	2.41	1	0	carbotricyclic compound; tertiary amine	adrenergic uptake inhibitor; antidepressant; environmental contaminant; tropomyosin-related kinase B receptor agonist; xenobiotic
4-cresol 4-cresol: RN given refers to parent cpd. p-cresol : A cresol that consists of toluene substituted by a hydroxy group at position 4. It is a metabolite of aromatic amino acid metabolism produced by intestinal microflora in humans and animals.	3.91	2	1	cresol	Escherichia coli metabolite; human metabolite; uremic toxin
cyclobenzaprine cyclobenzaprine: RN given refers to parent cpd; Lisseril is synonymous for HCl; structure. cyclobenzaprine : 5-Methylidene-5H-dibenzo[a,d]cycloheptene in which one of the hydrogens of the methylidene group is substituted by a 2-(dimethylamino)ethyl group. A centrally acting skeletal muscle relaxant, it is used as its hydrochloride salt in the symptomatic treatment of painful muscle spasm.	2.41	1	0	carbotricyclic compound	antidepressant; muscle relaxant; tranquilizing drug
haloperidol Haloperidol: A phenyl-piperidinyl-butyrophenone that is used primarily to treat SCHIZOPHRENIA and other PSYCHOSES. It is also used in schizoaffective disorder, DELUSIONAL DISORDERS, ballism, and TOURETTE SYNDROME (a drug of choice) and occasionally as adjunctive therapy in INTELLECTUAL DISABILITY and the chorea of HUNTINGTON DISEASE. It is a potent antiemetic and is used in the treatment of intractable HICCUPS. (From AMA Drug Evaluations Annual, 1994, p279). haloperidol : A compound composed of a central piperidine structure with hydroxy and p-chlorophenyl substituents at position 4 and an N-linked p-fluorobutyrophenone moiety.	1.95	1	0	aromatic ketone; hydroxypiperidine; monochlorobenzenes; organofluorine compound; tertiary alcohol	antidyskinesia agent; antiemetic; dopaminergic antagonist; first generation antipsychotic; serotonergic antagonist
4-phenylbutyric acid 4-phenylbutyric acid: RN refers to the parent cpd. 4-phenylbutyric acid : A monocarboxylic acid the structure of which is that of butyric acid substituted with a phenyl group at C-4. It is a histone deacetylase inhibitor that displays anticancer activity. It inhibits cell proliferation, invasion and migration and induces apoptosis in glioma cells. It also inhibits protein isoprenylation, depletes plasma glutamine, increases production of foetal haemoglobin through transcriptional activation of the gamma-globin gene and affects hPPARgamma activation.	6.39	5	3	monocarboxylic acid	antineoplastic agent; apoptosis inducer; EC 3.5.1.98 (histone deacetylase) inhibitor; prodrug
thioridazine Thioridazine: A phenothiazine antipsychotic used in the management of PHYCOSES, including SCHIZOPHRENIA.. thioridazine : A phenothiazine derivative having a methylsulfanyl subsitituent at the 2-position and a (1-methylpiperidin-2-yl)ethyl] group at the N-10 position.	1.95	1	0	phenothiazines; piperidines	alpha-adrenergic antagonist; dopaminergic antagonist; EC 1.8.1.12 (trypanothione-disulfide reductase) inhibitor; EC 3.4.21.26 (prolyl oligopeptidase) inhibitor; first generation antipsychotic; H1-receptor antagonist; serotonergic antagonist
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.	1.96	1	0	alanine zwitterion; alanine; L-alpha-amino acid; proteinogenic amino acid; pyruvate family amino acid	EC 4.3.1.15 (diaminopropionate ammonia-lyase) inhibitor; fundamental metabolite
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.	14.88	84	15	amino 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.	1.96	1	0	aspartate 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
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.	2.25	1	0	amino 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
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.	2.37	2	0	amino 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
ornithine Ornithine: An amino acid produced in the urea cycle by the splitting off of urea from arginine.. ornithine : An alpha-amino acid that is pentanoic acid bearing two amino substituents at positions 2 and 5.	2.52	2	0	non-proteinogenic L-alpha-amino acid; ornithine	algal metabolite; hepatoprotective agent; mouse 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.	3.23	1	0	amino 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
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.	2.46	2	0	amino 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
tryptophan Tryptophan: An essential amino acid that is necessary for normal growth in infants and for NITROGEN balance in adults. It is a precursor of INDOLE ALKALOIDS in plants. It is a precursor of SEROTONIN (hence its use as an antidepressant and sleep aid). It can be a precursor to NIACIN, albeit inefficiently, in mammals.. tryptophan : An alpha-amino acid that is alanine bearing an indol-3-yl substituent at position 3.	2.57	2	0	erythrose 4-phosphate/phosphoenolpyruvate family amino acid; L-alpha-amino acid zwitterion; L-alpha-amino acid; proteinogenic amino acid; tryptophan zwitterion; tryptophan	antidepressant; 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.	2.25	1	0	aspartate 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.	1.98	1	0	arginine; glutamine family amino acid; L-alpha-amino acid; proteinogenic amino acid	biomarker; Escherichia coli metabolite; micronutrient; mouse metabolite; nutraceutical
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.	2.25	1	0	branched-chain saturated fatty acid; fatty acid 4:0; methyl-branched fatty acid	Daphnia magna metabolite; plant metabolite; volatile oil component
uridine diphosphate glucose Uridine Diphosphate Glucose: A key intermediate in carbohydrate metabolism. Serves as a precursor of glycogen, can be metabolized into UDPgalactose and UDPglucuronic acid which can then be incorporated into polysaccharides as galactose and glucuronic acid. Also serves as a precursor of sucrose lipopolysaccharides, and glycosphingolipids.. UDP-alpha-D-glucose : The alpha-anomer of UDP-alpha-D-glucose. It is used in nucleotide sugars metabolism.	1.98	1	0	UDP-D-glucose	fundamental metabolite
citrulline citrulline : The parent compound of the citrulline class consisting of ornithine having a carbamoyl group at the N(5)-position.	1.96	1	0	amino 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
2-piperidone 2-piperidone: structure given in first source. piperidin-2-one : A delta-lactam that is piperidine which is substituted by an oxo group at position 2.	2	1	0	delta-lactam; piperidones	EC 1.2.1.88 (L-glutamate gamma-semialdehyde dehydrogenase) inhibitor
pseudouridine [no description available]	2.21	1	0	pseudouridines	fundamental metabolite
deuterium Deuterium: The stable isotope of hydrogen. It has one neutron and one proton in the nucleus.	3.8	2	0	dihydrogen
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.	2.17	1	0	benzenes; phenyl acetates
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.	3.78	3	0	glutamic acid; glutamine family amino acid; L-alpha-amino acid; proteinogenic amino acid	Escherichia coli metabolite; ferroptosis inducer; micronutrient; mouse metabolite; neurotransmitter; nutraceutical
antineoplaston a10 antineoplaston A10: peptide analogue isolated from human urine	2	1	0
acetaminophen glucuronide acetaminophen glucuronide: acetaminophen metabolite in rats. acetaminophen O-beta-D-glucosiduronic acid : A beta-D-glucosiduronic acid that is the O-glucuronide of paracetamol (acetaminophen).	4.08	3	0	beta-D-glucosiduronic acid	drug metabolite
n-acetylthreonine N-acetyl-L-threonine : A N-acetyl-L-amino acid that is the N-acetyl derivative of L-threonine.	2.21	1	0	L-threonine derivative; N-acetyl-L-amino acid
indican [no description available]	5.49	7	2	beta-D-glucoside; exopolysaccharide; indolyl carbohydrate
4-cresol sulfate p-cresol sulfate : An aryl sulfate that is p-cresol in which the phenolic hydrogen has been replaced by a sulfo group.	5.34	6	2	aryl sulfate	gut flora metabolite; human metabolite; uremic toxin
glycerol phenylbutyrate glycerol phenylbutyrate: for treating urea cycle disorders	9.2	12	4	triglyceride
ornithine phenylacetate [no description available]	2.52	2	0
natriuretic peptide, brain Natriuretic Peptide, Brain: A PEPTIDE that is secreted by the BRAIN and the HEART ATRIA, stored mainly in cardiac ventricular MYOCARDIUM. It can cause NATRIURESIS; DIURESIS; VASODILATION; and inhibits secretion of RENIN and ALDOSTERONE. It improves heart function. It contains 32 AMINO ACIDS.	4.4	2	1	polypeptide
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.	2.17	1	0

Condition	Relationship Strength	Studies	Trials
Affective Disorders [description not available]	3.23	1	0
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).	3.23	1	0
Mood Disorders Those disorders that have a disturbance in mood as their predominant feature.	3.23	1	0
Chronic Illness [description not available]	2.41	1	0
Cardiac Failure [description not available]	6.94	11	4
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).	2.41	1	0
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.	6.94	11	4
Kidney Diseases Pathological processes of the KIDNEY or its component tissues.	2.59	2	0
Diabetes Mellitus, Adult-Onset [description not available]	2.94	3	0
Cardiovascular Stroke [description not available]	2.72	2	0
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.	2.94	3	0
Myocardial Infarction NECROSIS of the MYOCARDIUM caused by an obstruction of the blood supply to the heart (CORONARY CIRCULATION).	2.72	2	0
Asymmetric Diabetic Proximal Motor Neuropathy [description not available]	2.6	1	0
Polyneuropathy, Acquired [description not available]	2.6	1	0
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)	2.6	1	0
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.	2.6	1	0
Disbacteriosis [description not available]	3.52	4	0
Left Ventricular Dysfunction [description not available]	6.08	4	4
Ventricular Dysfunction, Left A condition in which the LEFT VENTRICLE of the heart was functionally impaired. This condition usually leads to HEART FAILURE; MYOCARDIAL INFARCTION; and other cardiovascular complications. Diagnosis is made by measuring the diminished ejection fraction and a depressed level of motility of the left ventricular wall.	6.08	4	4
Cardiovascular Diseases Pathological conditions involving the CARDIOVASCULAR SYSTEM including the HEART; the BLOOD VESSELS; or the PERICARDIUM.	8.02	9	1
Arrhythmia [description not available]	7.6	1	0
Disease Models, Animal Naturally-occurring or experimentally-induced animal diseases with pathological processes analogous to human diseases.	4.06	4	0
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.	2.6	1	0
Encephalopathy, Traumatic [description not available]	2.6	1	0
Brain Injuries, Traumatic A form of acquired brain injury which occurs when a sudden trauma causes damage to the brain.	2.6	1	0
Colitis, Granulomatous [description not available]	2.6	1	0
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.	7.6	1	0
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.	2.6	1	0
Chronic Kidney Diseases [description not available]	5.37	6	0
Vascular Calcification Deposition of calcium into the blood vessel structures. Excessive calcification of the vessels are associated with ATHEROSCLEROTIC PLAQUES formation particularly after MYOCARDIAL INFARCTION (see MONCKEBERG MEDIAL CALCIFIC SCLEROSIS) and chronic kidney diseases which in turn increase VASCULAR STIFFNESS.	2.6	1	0
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)	5.37	6	0
Inborn Urea Cycle Disorder [description not available]	12.49	47	7
Urea Cycle Disorders, Inborn Rare congenital metabolism disorders of the urea cycle. The disorders are due to mutations that result in complete (neonatal onset) or partial (childhood or adult onset) inactivity of an enzyme, involved in the urea cycle. Neonatal onset results in clinical features that include irritability, vomiting, lethargy, seizures, NEONATAL HYPOTONIA; RESPIRATORY ALKALOSIS; HYPERAMMONEMIA; coma, and death. Survivors of the neonatal onset and childhood/adult onset disorders share common risks for ENCEPHALOPATHIES, METABOLIC, INBORN; and RESPIRATORY ALKALOSIS due to HYPERAMMONEMIA.	12.49	47	7
Birth Weight The mass or quantity of heaviness of an individual at BIRTH. It is expressed by units of pounds or kilograms.	2.25	1	0
Insulin Sensitivity [description not available]	2.25	1	0
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.	2.25	1	0
Pregnancy The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.	2.25	1	0
Apoplexy [description not available]	2.25	1	0
Blood Clot [description not available]	3.57	2	0
Cardiac Arrest, Sudden [description not available]	2.25	1	0
Thrombosis Formation and development of a thrombus or blood clot in the blood vessel.	3.57	2	0
Death, Sudden, Cardiac Unexpected rapid natural death due to cardiovascular collapse within one hour of initial symptoms. It is usually caused by the worsening of existing heart diseases. The sudden onset of symptoms, such as CHEST PAIN and CARDIAC ARRHYTHMIAS, particularly VENTRICULAR TACHYCARDIA, can lead to the loss of consciousness and cardiac arrest followed by biological death. (from Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine, 7th ed., 2005)	2.25	1	0
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)	2.25	1	0
Arteriosclerosis, Coronary [description not available]	3.6	2	0
Coronary Artery Disease Pathological processes of CORONARY ARTERIES that may derive from a congenital abnormality, atherosclerotic, or non-atherosclerotic cause.	3.6	2	0
Atherogenesis [description not available]	3.17	1	0
Angiitis [description not available]	3.17	1	0
Vasculitis Inflammation of any one of the blood vessels, including the ARTERIES; VEINS; and rest of the vasculature system in the body.	3.17	1	0
Atherosclerosis A thickening and loss of elasticity of the walls of ARTERIES that occurs with formation of ATHEROSCLEROTIC PLAQUES within the ARTERIAL INTIMA.	3.17	1	0
Coronary Artery Stenosis [description not available]	2.31	1	0
Atheroma [description not available]	2.31	1	0
Coronary Stenosis Narrowing or constriction of a coronary artery.	2.31	1	0
Chronic Kidney Failure [description not available]	4.34	4	1
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.	4.34	4	1
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.	4.99	3	1
Deficiency Disease, Ornithine Carbamoyltransferase [description not available]	2.43	2	0
Arginino Succinase Deficiency [description not available]	2.15	1	0
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)	2.43	2	0
Argininosuccinic Aciduria Rare autosomal recessive disorder of the urea cycle which leads to the accumulation of argininosuccinic acid in body fluids and severe HYPERAMMONEMIA. Clinical features of the neonatal onset of the disorder include poor feeding, vomiting, lethargy, seizures, tachypnea, coma, and death. Later onset results in milder set of clinical features including vomiting, failure to thrive, irritability, behavioral problems, or psychomotor retardation. Mutations in the ARGININOSUCCINATE LYASE gene cause the disorder.	2.15	1	0
Acute Hepatic Failure [description not available]	2.17	1	0
Liver Failure, Acute A form of rapid-onset LIVER FAILURE, also known as fulminant hepatic failure, caused by severe liver injury or massive loss of HEPATOCYTES. It is characterized by sudden development of liver dysfunction and JAUNDICE. Acute liver failure may progress to exhibit cerebral dysfunction even HEPATIC COMA depending on the etiology that includes hepatic ISCHEMIA, drug toxicity, malignant infiltration, and viral hepatitis such as post-transfusion HEPATITIS B and HEPATITIS C.	2.17	1	0
Hyperammonemia Elevated level of AMMONIA in the blood. It is a sign of defective CATABOLISM of AMINO ACIDS or ammonia to UREA.	7.86	6	4
Encephalopathy, Toxic [description not available]	2.17	1	0
Benign Neoplasms [description not available]	11.73	42	4
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.	11.73	42	4
Aging The gradual irreversible changes in structure and function of an organism that occur as a result of the passage of time.	2.08	1	0
Orphan Diseases Rare diseases that have not been well studied.	2.08	1	0
Encephalopathy, Hepatic [description not available]	11.8	41	4
Hepatic Encephalopathy A syndrome characterized by central nervous system dysfunction in association with LIVER FAILURE, including portal-systemic shunts. Clinical features include lethargy and CONFUSION (frequently progressing to COMA); ASTERIXIS; NYSTAGMUS, PATHOLOGIC; brisk oculovestibular reflexes; decorticate and decerebrate posturing; MUSCLE SPASTICITY; and bilateral extensor plantar reflexes (see REFLEX, BABINSKI). ELECTROENCEPHALOGRAPHY may demonstrate triphasic waves. (From Adams et al., Principles of Neurology, 6th ed, pp1117-20; Plum & Posner, Diagnosis of Stupor and Coma, 3rd ed, p222-5)	11.8	41	4
Adverse Drug Event [description not available]	9.01	36	0
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.	9.01	36	0
Diseases in Twins Disorders affecting TWINS, one or both, at any age.	2.11	1	0
Diabetic Glomerulosclerosis [description not available]	2.11	1	0
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.	2.11	1	0
Disease Exacerbation [description not available]	2.13	1	0
Adenocarcinoma, Basal Cell [description not available]	2.13	1	0
Condition, Preneoplastic [description not available]	2.13	1	0
Cancer of Stomach [description not available]	2.13	1	0
Infections, Helicobacter [description not available]	2.13	1	0
Adenocarcinoma A malignant epithelial tumor with a glandular organization.	2.13	1	0
Gastritis Inflammation of the GASTRIC MUCOSA, a lesion observed in a number of unrelated disorders.	2.13	1	0
Precancerous Conditions Pathological conditions that tend eventually to become malignant.	2.13	1	0
Stomach Neoplasms Tumors or cancer of the STOMACH.	2.13	1	0
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.	2.13	1	0
Bladder Pain Syndrome [description not available]	2.05	1	0
Cystitis, Interstitial A condition with recurring discomfort or pain in the URINARY BLADDER and the surrounding pelvic region without an identifiable disease. Severity of pain in interstitial cystitis varies greatly and often is accompanied by increased urination frequency and urgency.	2.05	1	0
Cirrhosis, Liver [description not available]	7.48	4	4
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.	7.48	4	4
Encephalomyelitis, Inflammatory [description not available]	1.93	1	0
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.	1.93	1	0
Childhood Torsion Disease [description not available]	1.96	1	0
Chronic Motor and Vocal Tic Disorder [description not available]	1.95	1	0
Bodily Distress Disorder [description not available]	1.95	1	0
Symptom Cluster [description not available]	1.95	1	0
Tourette Syndrome A neuropsychological disorder related to alterations in DOPAMINE metabolism and neurotransmission involving frontal-subcortical neuronal circuits. Both multiple motor and one or more vocal tics need to be present with TICS occurring many times a day, nearly daily, over a period of more than one year. The onset is before age 18 and the disturbance is not due to direct physiological effects of a substance or another medical condition. The disturbance causes marked distress or significant impairment in social, occupational, or other important areas of functioning. (From DSM-IV, 1994; Neurol Clin 1997 May;15(2):357-79)	1.95	1	0
Syndrome A characteristic symptom complex.	1.95	1	0
Amino Acid Metabolism Disorders, Inborn [description not available]	2.37	2	0
Hyperactivity, Motor [description not available]	1.95	1	0
Autoimmune Diabetes [description not available]	2.39	2	0
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.	2.39	2	0
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)	2.39	2	0
Hepatocellular Carcinoma [description not available]	1.98	1	0
Carcinoma, Hepatocellular A primary malignant neoplasm of epithelial liver cells. It ranges from a well-differentiated tumor with EPITHELIAL CELLS indistinguishable from normal HEPATOCYTES to a poorly differentiated neoplasm. The cells may be uniform or markedly pleomorphic, or form GIANT CELLS. Several classification schemes have been suggested.	1.98	1	0
Hyperglycemia, Postprandial Abnormally high BLOOD GLUCOSE level after a meal.	1.98	1	0
Hyperglycemia Abnormally high BLOOD GLUCOSE level.	1.98	1	0
Glial Cell Tumors [description not available]	2	1	0
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)	2	1	0
Anasarca [description not available]	4.31	1	1
Lassitude [description not available]	5.27	2	2
Nervous System Disorders [description not available]	4.31	1	1
Emesis [description not available]	4.31	1	1
Edema Abnormal fluid accumulation in TISSUES or body cavities. Most cases of edema are present under the SKIN in SUBCUTANEOUS TISSUE.	4.31	1	1
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.	5.27	2	2
Hypocalcemia Reduction of the blood calcium below normal. Manifestations include hyperactive deep tendon reflexes, Chvostek's sign, muscle and abdominal cramps, and carpopedal spasm. (Dorland, 27th ed)	4.31	1	1
Nausea An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses.	5.27	2	2
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.	4.31	1	1
Vomiting The forcible expulsion of the contents of the STOMACH through the MOUTH.	4.31	1	1
Daytime Sleepiness [description not available]	3.39	1	1
Hypokalemia Abnormally low potassium concentration in the blood. It may result from potassium loss by renal secretion or by the gastrointestinal route, as by vomiting or diarrhea. It may be manifested clinically by neuromuscular disorders ranging from weakness to paralysis, by electrocardiographic abnormalities (depression of the T wave and elevation of the U wave), by renal disease, and by gastrointestinal disorders. (Dorland, 27th ed)	3.39	1	1
Cancer of Prostate [description not available]	3.39	1	1
Disorders of Excessive Somnolence Disorders characterized by hypersomnolence during normal waking hours that may impair cognitive functioning. Subtypes include primary hypersomnia disorders (e.g., IDIOPATHIC HYPERSOMNOLENCE; NARCOLEPSY; and KLEINE-LEVIN SYNDROME) and secondary hypersomnia disorders where excessive somnolence can be attributed to a known cause (e.g., drug affect, MENTAL DISORDERS, and SLEEP APNEA SYNDROME). (From J Neurol Sci 1998 Jan 8;153(2):192-202; Thorpy, Principles and Practice of Sleep Medicine, 2nd ed, p320)	3.39	1	1
Neutropenia A decrease in the number of NEUTROPHILS found in the blood.	3.39	1	1
Prostatic Neoplasms Tumors or cancer of the PROSTATE.	3.39	1	1
Inborn Errors of Metabolism [description not available]	1.97	1	0
Metabolism, Inborn Errors Errors in metabolic processes resulting from inborn genetic mutations that are inherited or acquired in utero.	1.97	1	0

phenylacetylglutamine

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