Alpha-ketobutyric acid

α-Ketobutyric acid
CAS number 600-18-0 YesY
PubChem 58
ChemSpider 57 YesY
DrugBank DB04553
KEGG C00109 YesY
MeSH Alpha-ketobutyric+acid
ChEBI CHEBI:30831 YesY
Jmol-3D images Image 1
Molecular formula C4H6O3
Molar mass 102.089 g/mol
 YesY (verify) (what is: YesY/N?)
Except where noted otherwise, data are given for materials in their standard state (at 25 °C, 100 kPa)
Infobox references

α-Ketobutyric acid is a product of the lysis of cystathionine.

It is also one of the degradation products of threonine, produced by the catabolism of the amino acid by threonine dehydratase. It is also produced by the degradation of homocysteine and the metabolism of methionine.

α-Ketobutyric acid is transported into the mitochondrial matrix, where it is converted to propionyl-CoA by Branched-chain alpha-keto acid dehydrogenase complex. Further mitochondrial reactions produce succinyl CoA. This is first through the enzyme mitochondria Propionyl-CoA carboxylase with Biotin as a cofactor to produce S-methylmalonyl-CoA. This is subsequently converted to R-methylmalonyl-CoA by mitochondrial Methylmalonyl-CoA epimerase. Finally, mitochondrial Methylmalonyl-CoA mutase with cofactor Adenosylcobalamin produces succinyl-CoA which enters the citric acid cycle.[1]

Conversion in sotolon in French Vin jaune

Vin jaune is marked by the formation of sotolon from alpha-ketobutyric acid.[2][3]

See also