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 functions published in J. Biol. Chem.

PMID	Journal	Published Date	Author	Title
9748245	J. Biol. Chem.	1998	Dickinson JR et al.	An investigation of the metabolism of valine to isobutyl alcohol in Saccharomyces cerevisiae.
17329260	J. Biol. Chem.	2007	Li J et al.	The two active sites in human branched-chain alpha-keto acid dehydrogenase operate independently without an obligatory alternating-site mechanism.
11448970	J. Biol. Chem.	2001	Wynn RM et al.	Biochemical basis of type IB (E1beta ) mutations in maple syrup urine disease. A prevalent allele in patients from the Druze kindred in Israel.
11279000	J. Biol. Chem.	2001	Lee HJ et al.	Alteration of the co-substrate selectivity of deacetoxycephalosporin C synthase. The role of arginine 258.
11069910	J. Biol. Chem.	2001	Wynn RM et al.	Roles of active site and novel K+ ion-binding site residues in human mitochondrial branched-chain alpha-ketoacid decarboxylase/dehydrogenase.