Showing chemical card for 3-methyl-2-oxobutanoate (CFc000337585)
Record Information
Version
1.0
Creation Date
Update Date
2022-09-14 05:31:27 UTC
Chemfont ID
CFc000337585
Molecule Identification
Common Name
3-methyl-2-oxobutanoate
Definition
Alpha-ketoisovaleric acid, also known as alpha-oxoisovalerate or 3-methyl-2-oxobutanoate, belongs to short-chain keto acids and derivatives class of compounds. Those are keto acids with an alkyl chain the contains less than 6 carbon atoms. Thus, alpha-ketoisovaleric acid is considered to be a fatty acid lipid molecule. Alpha-ketoisovaleric acid is soluble (in water) and a weakly acidic compound (based on its pKa). Alpha-ketoisovaleric acid can be found in a number of food items such as kale, burdock, ginseng, and yellow zucchini, which makes alpha-ketoisovaleric acid a potential biomarker for the consumption of these food products. Alpha-ketoisovaleric acid can be found primarily in most biofluids, including blood, cerebrospinal fluid (CSF), saliva, and urine, as well as in human most tissues tissue. Alpha-ketoisovaleric acid exists in all living species, ranging from bacteria to humans. In humans, alpha-ketoisovaleric acid is involved in the valine, leucine and isoleucine degradation. Alpha-ketoisovaleric acid is also involved in several metabolic disorders, some of which include beta-ketothiolase deficiency, 3-methylglutaconic aciduria type IV, 3-methylglutaconic aciduria type I, and isobutyryl-coa dehydrogenase deficiency. Moreover, alpha-ketoisovaleric acid is found to be associated with maple syrup urine disease. Alpha-ketoisovaleric acid is a non-carcinogenic (not listed by IARC) potentially toxic compound.
Belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
