Recombinant Human Isovaleryl-CoA dehydrogenase, mitochondrial (IVD)

Uniprot Id

P26440

Target Species

Human

Target Name

IVD

Target Full Name

Isovaleryl-CoA dehydrogenase, mitochondrial

Target Function

Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway. To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as pentanoyl-CoA, hexenoyl-CoA and butenoyl-CoA.

Target Involvement

Isovaleric acidemia (IVA)

Target Subcellular Location

Mitochondrion matrix.

Target Protein Families

Acyl-CoA dehydrogenase family

Target Synonyms

ACAD2; FLJ12715; FLJ34849; Isovaleryl CoA dehydrogenase; Isovaleryl CoA dehydrogenase, mitochondrial ; Isovaleryl Coenzyme A dehydrogenase; Isovaleryl-CoA dehydrogenase; IVD; IVD_HUMAN; mitochondrial

Target Background

Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript variants have been found for this gene.