Function
2-oxoisovalerate dehydrogenase (OIVD) or branched-chain α-keto acid decarboxylase is part of a multienzyme complex metabolizing pyruvate, 2-oxogluterate and branched-chain 2-oxo acids. The multienzyme complex (BCKDC) contains three catalytic components: OIVD, lipoamide acyltransferase and lipoamide dehydrogenase, a kinase and a phosphatase. The active site of OIVD contains the cofactor thiamin diphosphate (ThDP)[1].
Disease
Mutations in OIVD result in maple syrup urine disease characterized by mental and physical retardation and feeding problems[2].
Structural highlights
. The structure of the phosphorylated human OIVD shows the (water molecules shown as red spheres). Phosphorylation of Ser292 of the α subunit interferes with ThDP binding[3].
3D structures of 2-oxoisovalerate dehydrogenase
2-oxoisovalerate dehydrogenase 3D structures
