Page last updated: 2024-08-26

3-methylglutaconic acid and leucine

3-methylglutaconic acid has been researched along with leucine in 13 studies

Research

Studies (13)

TimeframeStudies, this research(%)All Research%
pre-19903 (23.08)18.7374
1990's6 (46.15)18.2507
2000's4 (30.77)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Gibson, KM; Lee, CF; Wappner, RS1
Chalmers, RA; Halliday, D; Thompson, GN1
Beemer, FA; Bruinvis, L; Duran, M; Ketting, D; Tibosch, AS; Wadman, SK1
Hagberg, B; Hjalmarson, O; Lindstedt, S; Ransnäs, L; Steen, G1
Berry, HK; Denton, MD; Norman, EJ1
Deufel, T; Duran, M; Endres, W; Gibson, KM; Hadorn, HB; Ibel, H; Kennaway, NG; Paetzke, I1
Yoshida, I2
De Meirleir, L; Desprechins, B; Erasmus, E; Gerlo, E; Gibson, KM; Jooste, S; Mienie, LJ; Wappner, RS1
Bode, HB; Gaitatzis, N; Mahmud, T; Müller, R; Wan, E; Wen, KW; Wenzel, SC1
Gunay-Aygun, M1
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
Buckel, W; Hoffmann, GF; Liesert, M; Mack, M; Peters, V; Schniegler-Mattox, U; Zschocke, J1

Reviews

3 review(s) available for 3-methylglutaconic acid and leucine

ArticleYear
[3-Methylglutaconyl-CoA hydratase deficiency].
    Ryoikibetsu shokogun shirizu, 1998, Issue:18 Pt 1

    Topics: Amino Acid Metabolism, Inborn Errors; Diagnosis, Differential; Glutarates; Humans; Hydro-Lyases; Leucine; Prognosis

1998
[Methylglutaconic aciduria (normal hydratase, unspecified)].
    Ryoikibetsu shokogun shirizu, 1998, Issue:18 Pt 1

    Topics: Amino Acid Metabolism, Inborn Errors; Diagnosis, Differential; Glutarates; Humans; Leucine; Mevalonic Acid; Prognosis

1998
3-Methylglutaconic aciduria: a common biochemical marker in various syndromes with diverse clinical features.
    Molecular genetics and metabolism, 2005, Volume: 84, Issue:1

    Topics: Abnormalities, Multiple; Acetyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Glutarates; Humans; Hydro-Lyases; Leucine; Mitochondrial Diseases; Sterols

2005

Other Studies

10 other study(ies) available for 3-methylglutaconic acid and leucine

ArticleYear
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
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
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
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
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
Variable clinical presentation in three patients with 3-methylglutaconyl-coenzyme A hydratase deficiency.
    Journal of inherited metabolic disease, 1998, Volume: 21, Issue:6

    Topics: Adolescent; Brain; Developmental Disabilities; Female; Glutarates; Humans; Hydro-Lyases; Infant; Leucine; Magnetic Resonance Imaging; Male; Metabolism, Inborn Errors

1998
A biosynthetic pathway to isovaleryl-CoA in myxobacteria: the involvement of the mevalonate pathway.
    Chembiochem : a European journal of chemical biology, 2005, Volume: 6, Issue:2

    Topics: Acyl Coenzyme A; Animals; Glutarates; Leucine; Methacrylates; Mevalonic Acid; Molecular Structure; Myxococcales; Quinolines; Stigmatella aurantiaca; Thiazoles

2005
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
Biochemical characterization of human 3-methylglutaconyl-CoA hydratase and its role in leucine metabolism.
    The FEBS journal, 2006, Volume: 273, Issue:9

    Topics: Acyl Coenzyme A; Amino Acid Metabolism, Inborn Errors; Cells, Cultured; Cloning, Molecular; Escherichia coli; Fibroblasts; Glutarates; Humans; Hydro-Lyases; Kinetics; Leucine; Mutation; Substrate Specificity

2006