Compounds > 2-hydroxyisovaleric acid
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2-hydroxyisovaleric acid and BCKD Deficiency

2-hydroxyisovaleric acid has been researched along with BCKD Deficiency in 4 studies

Research

Studies (4)

TimeframeStudies, this research(%)All Research%
pre-19902 (50.00)18.7374
1990's1 (25.00)18.2507
2000's0 (0.00)29.6817
2010's1 (25.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Amaral, AU; Fernandes, CG; Leipnitz, G; Schuck, PF; Seminotti, B; Wajner, M1
Jakobs, C; Nyhan, WL; Sweetman, L1
Ckless, K; Schlottfeldt, JL; Wajner, M; Wannmacher, CM1
Hagenfeldt, L; Naglo, AS1

Other Studies

4 other study(ies) available for 2-hydroxyisovaleric acid and BCKD Deficiency

ArticleYear
Alpha-ketoisocaproic acid and leucine provoke mitochondrial bioenergetic dysfunction in rat brain.
    Brain research, 2010, Apr-09, Volume: 1324

    Topics: Animals; Brain; Central Nervous System Agents; Electron Transport; Homeostasis; Keto Acids; Ketoglutarate Dehydrogenase Complex; Leucine; Maple Syrup Urine Disease; Membrane Potential, Mitochondrial; Mitochondrial Diseases; Mitochondrial Swelling; NADP; Oxygen Consumption; Prosencephalon; Rats; Rats, Wistar; Valerates

2010
Hydroxy acid metabolites of branched-chain amino acids in amniotic fluid.
    Clinica chimica acta; international journal of clinical chemistry, 1984, Jul-16, Volume: 140, Issue:2

    Topics: Amino Acid Metabolism, Inborn Errors; Amino Acids, Branched-Chain; Amniotic Fluid; Caproates; Female; Gas Chromatography-Mass Spectrometry; Humans; Hydroxy Acids; Hydroxybutyrates; Maple Syrup Urine Disease; Methylmalonic Acid; Pentanoic Acids; Pregnancy; Prenatal Diagnosis; Valerates

1984
Immunosuppressive effects of organic acids accumulating in patients with maple syrup urine disease.
    Journal of inherited metabolic disease, 1995, Volume: 18, Issue:2

    Topics: Caproates; Cells, Cultured; Hemiterpenes; Humans; Keto Acids; Lymphocyte Activation; Maple Syrup Urine Disease; Mitogens; Pentanoic Acids; Phytohemagglutinins; Valerates

1995
New conjugated urinary metabolites in intermediate type maple syrup urine disease.
    Clinica chimica acta; international journal of clinical chemistry, 1987, Oct-30, Volume: 169, Issue:1

    Topics: Amino Acids; Amino Acids, Branched-Chain; Carboxylic Acids; Female; Gas Chromatography-Mass Spectrometry; Glycine; Humans; Infant; Lactates; Lactic Acid; Maple Syrup Urine Disease; Valerates

1987