3-Methyl-2-oxobutanoic acid

3-Methyl-2-oxobutanoic acid is a lipid of Fatty Acyls (FA) class. 3-methyl-2-oxobutanoic acid is associated with abnormalities such as Maple Syrup Urine Disease and Kidney Failure, Chronic. The involved functions are known as Phosphorylation, Citric Acid Cycle, inhibitors, Process and Metabolic Control. 3-methyl-2-oxobutanoic acid often locates in Mitochondria, BL21, Cytoplasm, Ribosomes and Head. The associated genes with 3-Methyl-2-oxobutanoic acid are Genome, Homologous Gene, Operon, Alleles and Oxidoreductase Gene. The related lipids are dimyristoylphosphatidylglycerol, 9-oxononanoic acid, Valerates and alpha-ketocaproic acid.

References related to abnormalities published in J. Biol. Chem.


PMIDJournalPublished DateAuthorTitle
11069910J. Biol. Chem.2001Wynn RM et al.Roles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase.