meglutol and 3-methylglutaric acid

meglutol has been researched along with 3-methylglutaric acid in 39 studies

Research

Studies (39)

Timeframe	Studies, this research(%)	All Research%
pre-1990	6 (15.38)	18.7374
1990's	11 (28.21)	18.2507
2000's	7 (17.95)	29.6817
2010's	13 (33.33)	24.3611
2020's	2 (5.13)	2.80

Authors

Authors	Studies
Kuhara, T; Kuwabara, S; Matsumoto, I; Saiki, K; Takabayashi, H	1
Chemke, J; Chitayat, D; Gibson, KM; Kronick, JB; Mamer, OA; McGill, JJ; Rosenblatt, B; Scriver, CR; Sweetman, L	1
Gibson, KM; Lee, CF; Wappner, RS	1
Ben-Ezra, D; Elpeleg, ON; Raz, J; Sheffer, RN; Zlotogora, J	1
Bachmann, C; Barth, PG; Dianzani, I; Gibson, KM; Hoffman, GF; Schrynemackers-Pitance, P; Schutgens, RB; Sherwood, WG; Stumpf, DA; Weismann, U	1
Cheatham, JP; Clark, BJ; Kelley, RI; Nigro, MA; Powell, BR; Sherwood, GW; Sladky, JT; Swisher, WP	1
Brown, GK; Haan, EA; Pitt, JJ; Scholem, RD; Wraith, JE	1
Lehnert, W; Scharf, J; Wendel, U	1
Beemer, FA; Bruinvis, L; Duran, M; Ketting, D; Tibosch, AS; Wadman, SK	1
Hagberg, B; Hjalmarson, O; Lindstedt, S; Ransnäs, L; Steen, G	1
Hammond, J; Wilcken, B	1
Berry, HK; Denton, MD; Norman, EJ	1
Costeff, H; Elpeleg, ON; Gibson, KM; Joseph, A; Shental, Y; Weitz, R	1
Lerman-Sagie, T	1
Deufel, T; Duran, M; Endres, W; Gibson, KM; Hadorn, HB; Ibel, H; Kennaway, NG; Paetzke, I	1
Conway, H; Mayne, PD; Roche, G; Walsh, R	1
Gutman, A; Harel, S; Lerman-Sagie, T; Michelson, M	1
Brandis, M; Ensenauer, R; Gibson, KM; Lehnert, W; Müller, CB; Schwab, KO	1
Bodamer, OA; Naviaux, RK; Scaglia, F; Shapira, SK; Sutton, VR; Vladutiu, GD; Vogel, H	1
Fok, TF; Hui, J; Lam, CW; Law, LK; Tang, NL	1
Engelke, UF; Kluijtmans, LA; Kremer, B; Loss, S; Loupatty, FJ; Morava, E; Moskau, D; van den Bergh, E; van der Graaf, M; Wanders, RJ; Wevers, RA	1
Barich, DH; Gorman, EM; Munson, EJ; Zell, MT	1
Amaral, AU; de Bortoli, G; Latini, A; Leipnitz, G; Schuck, PF; Seminotti, B; Solano, A; Wajner, M; Wannmacher, CM; Wyse, AT	1
Amaral, AU; Beskow, AP; da Silva, Lde B; Fernandes, CG; Leipnitz, G; Ribeiro, CA; Seminotti, B; Vargas, CR; Wajner, M; Zanatta, A	1
Harada, A; Kono, K; Sakanishi, Y; Yuba, E	1
Hickmann, FH; Ribeiro, CA; Wajner, M	1
Busanello, EN; da Rosa, MS; Fernandes, CG; Lagranha, VL; Leipnitz, G; Martell, RW; Pierozan, P; Seminotti, B; Wajner, M	1
Caruso, U; Cassanello, M; D'Onofrio, V; Enea, A; Garrone, G; Guala, G; Poma, F; Porta, F; Puccinelli, P; Santarelli, F; Spada, M	1
Anikster, Y; Ben-Zeev, B; Blumkin, L; Hassin-Baer, S; Lerman-Sagie, T; Lev, D; Schweiger, A; Sofer, S; Yahalom, G	1
da Rosa, MS; Damiani, AP; de Andrade, VM; Leipnitz, G; Longaretti, LM; Pereira, M; Scaini, G; Schuck, PF; Seminotti, B; Streck, EL; Wajner, M; Zapelini, HG	1
Colín-González, AL; Fernandes, CG; Quincozes-Santos, A; Rodrigues, MDN; Santamaria, A; Seminotti, B; Wajner, M	1
Colín-González, AL; de Lima, ME; Galván-Arzate, S; Leipnitz, G; Paz-Loyola, AL; Ribeiro, CA; Santamaría, A; Seminotti, B; Souza, DO; Wajner, M	1
da Rosa, MS; Grings, M; Leipnitz, G; Parmeggiani, B; Ribeiro, CA; Seminotti, B; Wajner, M	1
Acquaviva-Bourdain, C; Briand, G; Dobbelaere, D; Fontaine, M; Jissendi-Tchofo, P; Mention, K; Roland, D; Ultré, V; Vamecq, J	1
Lai, YR; Tsai, SW; Wu, TY	1
da Rosa, MS; da Rosa-Junior, NT; de Moura Alvorcem, L; Glänzel, NM; Leipnitz, G; Parmeggiani, B; Wajner, M	1
Balbueno Guerreiro, GB; de Moura Coelho, D; Delgado, CA; Diaz Jacques, CE; Manfredini, V; Sitta, A; Vargas, CR; Wajner, M	1
Jones, DE; Perez, L; Ryan, RO	1
Gornbein, JA; Koos, BJ	1

Reviews

1 review(s) available for meglutol and 3-methylglutaric acid

Article	Year
3-Methylglutaric acid in energy metabolism. Clinica chimica acta; international journal of clinical chemistry, 2020, Volume: 502 Topics: Energy Metabolism; Enoyl-CoA Hydratase; Humans; Meglutol; Mitochondria; Oxo-Acid-Lyases; RNA-Binding Proteins	2020

Other Studies

38 other study(ies) available for meglutol and 3-methylglutaric acid

Article	Year
3-Methylglutaconic aciduria in two adults. Clinica chimica acta; international journal of clinical chemistry, 1992, Apr-30, Volume: 207, Issue:1-2 Topics: Adult; Female; Glutarates; Humans; Meglutol; Pregnancy	1992
3-Methylglutaconic aciduria: a marker for as yet unspecified disorders and the relevance of prenatal diagnosis in a 'new' type ('type 4'). Journal of inherited metabolic disease, 1992, Volume: 15, Issue:2 Topics: Amniotic Fluid; Child; Female; Fibroblasts; Glutarates; Humans; Hydro-Lyases; Male; Meglutol; Metabolism, Inborn Errors; Pregnancy; Prenatal Diagnosis	1992
3-Methylglutaconyl-coenzyme-A hydratase deficiency: a new case. Journal of inherited metabolic disease, 1992, Volume: 15, Issue:3 Topics: Carnitine; Diseases in Twins; Fibroblasts; Glutarates; Humans; Hydro-Lyases; Infant; Leucine; Lymphocytes; Male; Meglutol	1992
Behr's syndrome and 3-methylglutaconic aciduria. American journal of ophthalmology, 1992, Oct-15, Volume: 114, Issue:4 Topics: Child; Child, Preschool; Female; Glutarates; Humans; Infant; Jews; Male; Meglutol; Nervous System Diseases; Nystagmus, Pathologic; Optic Atrophies, Hereditary; Syndrome; Visual Acuity	1992
Phenotypic heterogeneity in the syndromes of 3-methylglutaconic aciduria. The Journal of pediatrics, 1991, Volume: 118, Issue:6 Topics: Amino Acid Metabolism, Inborn Errors; Coenzyme A; Glutarates; Humans; Hydroxymethylglutaryl CoA Reductases; Meglutol; Phenotype	1991
X-linked dilated cardiomyopathy with neutropenia, growth retardation, and 3-methylglutaconic aciduria. The Journal of pediatrics, 1991, Volume: 119, Issue:5 Topics: Adult; Cardiomyopathy, Dilated; Child; Child, Preschool; Chromatography, High Pressure Liquid; Fumarates; Glutarates; Growth Disorders; Heart Failure; Humans; Male; Meglutol; Muscular Diseases; Neutropenia; Pedigree; Syndrome; X Chromosome	1991
Episodes of severe metabolic acidosis in a patient with 3-methylglutaconic aciduria. European journal of pediatrics, 1987, Volume: 146, Issue:5 Topics: Abnormalities, Multiple; Acidosis; Acidosis, Lactic; Child, Preschool; Citric Acid Cycle; Follow-Up Studies; Glutarates; Humans; Infant; Male; Meglutol; Virus Diseases	1987
3-Methylglutaconic and 3-methylglutaric aciduria in a patient with suspected 3-methylglutaconyl-CoA hydratase deficiency. European journal of pediatrics, 1985, Volume: 143, Issue:4 Topics: Acidosis; Amino Acid Metabolism, Inborn Errors; Female; Fibroblasts; Glutarates; Humans; Hydro-Lyases; Infant; Intellectual Disability; Meglutol; Muscle Hypotonia; Skin	1985
Inherited 3-methylglutaconic aciduria in two brothers--another defect of leucine metabolism. The Journal of pediatrics, 1982, Volume: 101, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Child; Child, Preschool; Glutarates; Humans; Hydro-Lyases; Leucine; Male; Meglutol; Speech Disorders; Valerates	1982
3-Methylglutaconic aciduria in two infants. Clinica chimica acta; international journal of clinical chemistry, 1983, Oct-31, Volume: 134, Issue:1-2 Topics: Creatinine; Female; Fibroblasts; Glutarates; Hemiterpenes; Humans; Hydroxymethylglutaryl-CoA Synthase; Infant; Leucine; Leukocytes; Meglutol; Metabolism, Inborn Errors; Pentanoic Acids	1983
3-hydroxy-3-methylglutaric, 3-methylglutaconic and 3-methylglutaric acids can be non-specific indicators of metabolic disease. Journal of inherited metabolic disease, 1984, Volume: 7 Suppl 2 Topics: Female; Glutarates; Humans; Infant; Male; Meglutol; Oxo-Acid-Lyases	1984
Gas-chromatographic/mass spectrometric detection of 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in double first cousins. Clinical chemistry, 1982, Volume: 28, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Female; Gas Chromatography-Mass Spectrometry; Glutarates; Humans; Hydroxy Acids; Infant; Leucine; Male; Meglutol; Oxo-Acid-Lyases; Valerates	1982
3-Methylglutaconic aciduria in the Iraqi-Jewish 'optic atrophy plus' (Costeff) syndrome. Developmental medicine and child neurology, 1994, Volume: 36, Issue:2 Topics: Adolescent; Adult; Child; Child, Preschool; Cognition Disorders; Female; Glutarates; Humans; Iraq; Jews; Male; Meglutol; Movement Disorders; Optic Atrophy; Syndrome	1994
Behr syndrome. Pediatric neurology, 1995, Volume: 12, Issue:1 Topics: Amino Acid Metabolism, Inborn Errors; Brain Diseases, Metabolic; Child; Glutarates; Humans; Meglutol; Optic Atrophy; Syndrome	1995
Multiple respiratory chain abnormalities associated with hypertrophic cardiomyopathy and 3-methylglutaconic aciduria. European journal of pediatrics, 1993, Volume: 152, Issue:8 Topics: Acidosis, Lactic; Cardiomyopathy, Hypertrophic; Electron Transport Complex IV; Glutarates; Humans; Hydro-Lyases; Infant; Leucine; Male; Meglutol; Metabolism, Inborn Errors; Mitochondrial Myopathies; Respiration Disorders	1993
What is the origin of 3-methylglutaconic acid? Journal of inherited metabolic disease, 1999, Volume: 22, Issue:3 Topics: Adolescent; Adrenal Hyperplasia, Congenital; Child; Child, Preschool; Female; Glutarates; Humans; Male; Meglutol	1999
Urinary organic acid screening in children with developmental language delay. Journal of inherited metabolic disease, 1999, Volume: 22, Issue:7 Topics: Child; Child, Preschool; Female; Fumarates; Glutarates; Humans; Language Development Disorders; Male; Malonates; Mass Screening; Meglutol	1999
3-Methylglutaconyl-CoA hydratase deficiency: a new patient with speech retardation as the leading sign. Journal of inherited metabolic disease, 2000, Volume: 23, Issue:4 Topics: Amino Acid Metabolism, Inborn Errors; Cells, Cultured; Diet, Protein-Restricted; Fibroblasts; Glutarates; Humans; Hydro-Lyases; Meglutol; Speech Disorders; Valerates	2000
Mitochondrial DNA depletion associated with partial complex II and IV deficiencies and 3-methylglutaconic aciduria. Journal of child neurology, 2001, Volume: 16, Issue:2 Topics: Biopsy; Blotting, Southern; Child, Preschool; DNA, Mitochondrial; Glutarates; Humans; Infant; Male; Meglutol; Mitochondrial Encephalomyopathies; Muscle, Skeletal	2001
3-methyglutaconic aciduria in a Chinese patient with glycogen storage disease Ib. Journal of inherited metabolic disease, 2003, Volume: 26, Issue:7 Topics: Adolescent; Blood Glucose; Cholesterol; Diet; DNA; Gluconeogenesis; Glycogen Storage Disease Type I; Humans; Lactic Acid; Male; Meglutol	2003
NMR spectroscopic studies on the late onset form of 3-methylglutaconic aciduria type I and other defects in leucine metabolism. NMR in biomedicine, 2006, Volume: 19, Issue:2 Topics: Amino Acid Metabolism, Inborn Errors; Brain; Female; Glutarates; Humans; Leucine; Magnetic Resonance Spectroscopy; Meglutol; Middle Aged; Valerates	2006
3-Methylglutaric acid as a 13C solid-state NMR standard. Solid state nuclear magnetic resonance, 2006, Volume: 30, Issue:3-4 Topics: Carbon Isotopes; Magnetic Resonance Spectroscopy; Meglutol; Reference Standards	2006
Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young rats. Life sciences, 2008, Mar-12, Volume: 82, Issue:11-12 Topics: Animals; Antioxidants; Cerebral Cortex; Glutarates; Glutathione; Lipid Peroxidation; Meglutol; Mitochondria; Molecular Structure; Oxidants; Oxidative Stress; Protein Carbonylation; Rats; Rats, Wistar; Superoxides; Thiobarbituric Acid Reactive Substances; Valerates	2008
Striatum is more vulnerable to oxidative damage induced by the metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency as compared to liver. International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 2009, Volume: 27, Issue:4 Topics: Animals; Corpus Striatum; Glutarates; Glutathione; Glycine; Humans; Liver; Male; Meglutol; Oxidative Stress; Oxo-Acid-Lyases; Rats; Rats, Wistar; Thiobarbituric Acid Reactive Substances; Valerates	2009
Carboxylated hyperbranched poly(glycidol)s for preparation of pH-sensitive liposomes. Journal of controlled release : official journal of the Controlled Release Society, 2011, Jan-05, Volume: 149, Issue:1 Topics: Animals; Arylsulfonates; Cell Line; Chemistry, Pharmaceutical; Cytoplasm; Drug Carriers; Flow Cytometry; Fluorescein-5-isothiocyanate; Hydrogen-Ion Concentration; Hydrophobic and Hydrophilic Interactions; Liposomes; Meglutol; Membrane Fusion; Mice; Microscopy, Confocal; Molecular Structure; Molecular Weight; Ovalbumin; Particle Size; Propylene Glycols; Solubility	2011
Neurochemical evidence that 3-methylglutaric acid inhibits synaptic Na+,K+-ATPase activity probably through oxidative damage in brain cortex of young rats. International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience, 2011, Volume: 29, Issue:1 Topics: Animals; Cerebral Cortex; Glutarates; Humans; Male; Meglutol; Mitochondria; Oxidation-Reduction; Oxidative Stress; Rats; Rats, Wistar; Sodium-Potassium-Exchanging ATPase; Synaptic Membranes; Valerates	2011
In vivo experimental evidence that the major metabolites accumulating in 3-hydroxy-3-methylglutaryl-CoA lyase deficiency induce oxidative stress in striatum of developing rats: a potential pathophysiological mechanism of striatal damage in this disorder. Molecular genetics and metabolism, 2013, Volume: 109, Issue:2 Topics: Acetyl-CoA C-Acetyltransferase; Amino Acid Metabolism, Inborn Errors; Animals; Antioxidants; Basal Ganglia; Catalase; Dizocilpine Maleate; Glutathione; Glutathione Peroxidase; Male; Malondialdehyde; Meglutol; Oxidative Stress; Protein Carbonylation; Rats; Rats, Wistar; Receptors, N-Methyl-D-Aspartate; Superoxide Dismutase; Vitamin E	2013
A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Italian journal of pediatrics, 2013, May-24, Volume: 39 Topics: Acetyl-CoA C-Acetyltransferase; Acidosis; Acute Disease; Amino Acid Metabolism, Inborn Errors; Glutarates; Glycine; Humans; Hypoglycemia; Infant, Newborn; Male; Meglutol	2013
The neuropsychological profile of patients with 3-methylglutaconic aciduria type III, Costeff syndrome. American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics, 2015, Volume: 168B, Issue:3 Topics: Adult; Chorea; Cognition Disorders; Executive Function; Female; Follow-Up Studies; Humans; Intelligence; Male; Meglutol; Memory, Short-Term; Metabolism, Inborn Errors; Middle Aged; Neuropsychological Tests; Optic Atrophy; Prognosis; Psychomotor Performance; Spastic Paraplegia, Hereditary; Young Adult	2015
Evidence that 3-hydroxy-3-methylglutaric and 3-methylglutaric acids induce DNA damage in rat striatum. Metabolic brain disease, 2015, Volume: 30, Issue:4 Topics: Animals; Corpus Striatum; DNA Damage; Dose-Response Relationship, Drug; Injections, Intraventricular; Male; Meglutol; Rats; Rats, Wistar	2015
Induction of a Proinflammatory Response in Cortical Astrocytes by the Major Metabolites Accumulating in HMG-CoA Lyase Deficiency: the Role of ERK Signaling Pathway in Cytokine Release. Molecular neurobiology, 2016, Volume: 53, Issue:6 Topics: Acetyl-CoA C-Acetyltransferase; Amino Acid Metabolism, Inborn Errors; Animals; Antioxidants; Astrocytes; Cell Shape; Cells, Cultured; Cerebral Cortex; Cytokines; Flavonoids; Gliosis; Heme Oxygenase (Decyclizing); Inflammation; Male; MAP Kinase Signaling System; Meglutol; Metabolome; Mitochondria; NF-kappa B; Protoporphyrins; Rats, Wistar; Reactive Oxygen Species	2016
Experimental Evidence that 3-Methylglutaric Acid Disturbs Mitochondrial Function and Induced Oxidative Stress in Rat Brain Synaptosomes: New Converging Mechanisms. Neurochemical research, 2016, Volume: 41, Issue:10 Topics: Animals; Antioxidants; Brain; Lipid Peroxidation; Male; Meglutol; Mitochondria; Oxidative Stress; Rats, Wistar; Reactive Oxygen Species; Receptors, Cannabinoid; Synaptosomes	2016
3-Hydroxy-3-methylglutaric and 3-methylglutaric acids impair redox status and energy production and transfer in rat heart: relevance for the pathophysiology of cardiac dysfunction in 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. Free radical research, 2016, Volume: 50, Issue:9 Topics: Animals; Disease Models, Animal; Heart Diseases; Humans; Meglutol; Oxidation-Reduction; Oxo-Acid-Lyases; Rats; Rats, Wistar	2016
Coupled brain and urine spectroscopy - in vivo metabolomic characterization of HMG-CoA lyase deficiency in 5 patients. Molecular genetics and metabolism, 2017, Volume: 121, Issue:2 Topics: Acetyl-CoA C-Acetyltransferase; Amino Acid Metabolism, Inborn Errors; Brain; Brain Chemistry; Cerebellum; Child; Child, Preschool; Female; Humans; Hydrogen-Ion Concentration; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Meglutol; Metabolomics; Proton Magnetic Resonance Spectroscopy; Urine; Valerates; White Matter	2017
CALB-Catalyzed Two-Step Alcoholytic Desymmetrization of 3-Methylglutaric Diazolides in MTBE. Applied biochemistry and biotechnology, 2018, Volume: 185, Issue:3 Topics: Alcohols; Catalysis; Enzymes, Immobilized; Fungal Proteins; Kinetics; Lipase; Meglutol; Methyl Ethers; Stereoisomerism; Substrate Specificity; Temperature; Thermodynamics	2018
Bezafibrate In Vivo Administration Prevents 3-Methylglutaric Acid-Induced Impairment of Redox Status, Mitochondrial Biogenesis, and Neural Injury in Brain of Developing Rats. Neurotoxicity research, 2019, Volume: 35, Issue:4 Topics: Animals; Bezafibrate; Brain Injuries; Caspase 3; Male; MAP Kinase Signaling System; Meglutol; Organelle Biogenesis; Oxidation-Reduction; Oxidative Stress; Rats, Wistar; Synaptophysin; tau Proteins	2019
Prevention by L-carnitine of DNA damage induced by 3-hydroxy-3-methylglutaric and 3-methylglutaric acids and experimental evidence of lipid and DNA damage in patients with 3-hydroxy-3-methylglutaric aciduria. Archives of biochemistry and biophysics, 2019, 06-15, Volume: 668 Topics: 8-Hydroxy-2'-Deoxyguanosine; Acetyl-CoA C-Acetyltransferase; Adolescent; Amino Acid Metabolism, Inborn Errors; Carnitine; Child; Child, Preschool; Dinoprost; DNA Damage; Guanine; Guanosine; Humans; Infant; Lipid Peroxidation; Meglutol	2019
Early pregnancy metabolites predict gestational diabetes mellitus: implications for fetal programming. American journal of obstetrics and gynecology, 2021, Volume: 224, Issue:2 Topics: Adult; Alanine; Arginine; Carnitine; Case-Control Studies; Diabetes, Gestational; Diet Therapy; Dopamine; Early Diagnosis; Epigenesis, Genetic; Female; Fetal Development; Gestational Age; Glucose Tolerance Test; Glucuronides; Humans; Hypoglycemic Agents; Lactones; Lysine; Meglutol; Metabolomics; Neopterin; Orotic Acid; Phenols; Pregnancy; Ribonucleosides; Sulfides	2021