meglutol and 3-methylglutaconic acid

meglutol has been researched along with 3-methylglutaconic acid in 29 studies

Research

Studies (29)

TimeframeStudies, this research(%)All Research%
pre-19908 (27.59)18.7374
1990's14 (48.28)18.2507
2000's5 (17.24)29.6817
2010's2 (6.90)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Kuhara, T; Kuwabara, S; Matsumoto, I; Saiki, K; Takabayashi, H1
Chemke, J; Chitayat, D; Gibson, KM; Kronick, JB; Mamer, OA; McGill, JJ; Rosenblatt, B; Scriver, CR; Sweetman, L1
Gibson, KM; Lee, CF; Wappner, RS1
Ben-Ezra, D; Elpeleg, ON; Raz, J; Sheffer, RN; Zlotogora, J1
Chalmers, RA; Halliday, D; Thompson, GN1
Bachmann, C; Barth, PG; Dianzani, I; Gibson, KM; Hoffman, GF; Schrynemackers-Pitance, P; Schutgens, RB; Sherwood, WG; Stumpf, DA; Weismann, U1
Cheatham, JP; Clark, BJ; Kelley, RI; Nigro, MA; Powell, BR; Sherwood, GW; Sladky, JT; Swisher, WP1
Buchanan, DN; Thoene, JG1
Brown, GK; Haan, EA; Pitt, JJ; Scholem, RD; Wraith, JE1
Gibson, KM; Narisawa, K; Nyhan, WL; Sweetman, L1
Lehnert, W; Scharf, J; Wendel, U1
Beemer, FA; Bruinvis, L; Duran, M; Ketting, D; Tibosch, AS; Wadman, SK1
Hagberg, B; Hjalmarson, O; Lindstedt, S; Ransnäs, L; Steen, G1
Hammond, J; Wilcken, B1
Berry, HK; Denton, MD; Norman, EJ1
Costeff, H; Elpeleg, ON; Gibson, KM; Joseph, A; Shental, Y; Weitz, R1
Lerman-Sagie, T1
Barone, R; Fiumara, A; Nigro, F; Pavone, L; Ribes, A1
Deufel, T; Duran, M; Endres, W; Gibson, KM; Hadorn, HB; Ibel, H; Kennaway, NG; Paetzke, I1
Kleinle, S; Krähenbühl, S; Liechti-Gallati, S; Ruesch, S; Schaffner, T; Weber, J; Wermuth, B; Wiesmann, UN1
Conway, H; Mayne, PD; Roche, G; Walsh, R1
Gutman, A; Harel, S; Lerman-Sagie, T; Michelson, M1
Brandis, M; Ensenauer, R; Gibson, KM; Lehnert, W; Müller, CB; Schwab, KO1
Bodamer, OA; Naviaux, RK; Scaglia, F; Shapira, SK; Sutton, VR; Vladutiu, GD; Vogel, H1
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, RA1
Amaral, AU; de Bortoli, G; Latini, A; Leipnitz, G; Schuck, PF; Seminotti, B; Solano, A; Wajner, M; Wannmacher, CM; Wyse, AT1
Amaral, AU; Beskow, AP; da Silva, Lde B; Fernandes, CG; Leipnitz, G; Ribeiro, CA; Seminotti, B; Vargas, CR; Wajner, M; Zanatta, A1
Hickmann, FH; Ribeiro, CA; Wajner, M1
Caruso, U; Cassanello, M; D'Onofrio, V; Enea, A; Garrone, G; Guala, G; Poma, F; Porta, F; Puccinelli, P; Santarelli, F; Spada, M1

Other Studies

29 other study(ies) available for meglutol and 3-methylglutaconic acid

ArticleYear
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
The contribution of protein catabolism to metabolic decompensation in 3-hydroxy-3-methylglutaric aciduria.
    European journal of pediatrics, 1990, Volume: 149, Issue:5

    Topics: Amino Acid Metabolism, Inborn Errors; Child; Diseases in Twins; Female; Glutarates; Humans; Leucine; Male; Meglutol; Oxo-Acid-Lyases; Phenylalanine; Proteins; Valerates

1990
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
Photodiode array detection for liquid chromatographic profiling of carboxylic acids in physiological fluids: 3-hydroxy-3-methylglutaric aciduria.
    Clinical chemistry, 1986, Volume: 32, Issue:1 Pt 1

    Topics: Chromatography, High Pressure Liquid; Gas Chromatography-Mass Spectrometry; Glutarates; Humans; Infant, Newborn; Meglutol; Metabolism, Inborn Errors; Spectrophotometry

1986
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-Methylglutaconyl-CoA hydratase, 3-methylcrotonyl-CoA carboxylase and 3-hydroxy-3-methylglutaryl-CoA lyase deficiencies: a coupled enzyme assay useful for their detection.
    Clinica chimica acta; international journal of clinical chemistry, 1989, Sep-15, Volume: 184, Issue:1

    Topics: 3-Hydroxybutyric Acid; Carbon-Carbon Ligases; Chromatography, High Pressure Liquid; Fibroblasts; Glutarates; Humans; Hydro-Lyases; Hydroxybutyrates; Ligases; Meglutol; Oxo-Acid-Lyases

1989
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
Pancreatitis and organic acidemias.
    The Journal of pediatrics, 1995, Volume: 126, Issue:5 Pt 1

    Topics: Acidosis; Acute Disease; Glutarates; Humans; Infant; Male; Meglutol; Pancreatitis

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
Combined 3-methylglutaconic and 3-hydroxy-3-methylglutaric aciduria with endocardial fibroelastosis and dilatative cardiomyopathy in male and female siblings with partial deficiency of complex II/III in fibroblasts.
    Enzyme & protein, 1996, Volume: 49, Issue:5-6

    Topics: Cardiomyopathy, Dilated; DNA, Mitochondrial; Electron Transport Complex II; Electron Transport Complex III; Endocardium; Female; Fibroblasts; Glutarates; Humans; Infant; Liver; Male; Meglutol; Mitochondria, Heart; Multienzyme Complexes; Muscle Hypotonia; Myocardium; Oxidoreductases; Point Mutation; Succinate Dehydrogenase

1996
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
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
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
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
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