Tento enzym, mitochondriální flavoprotein, katalyzuje oxidaci 3-methylbutanoyl-CoA na 3-methylbut-2-enoyl-CoA za použití FAD jako kofaktoru. Defekty enzymu jsou spojeny s isovalerovou acidémií (IVA).

A mitochondrial flavoprotein, this enzyme catalyzes the oxidation of 3-methylbutanoyl-CoA to 3-methylbut-2-enoyl-CoA using FAD as a cofactor. Defects in the enzyme, is associated with isovaleric acidemia (IVA).

DUI

D050769 MeSH Prohlížeč

CUI

M0076387

Předchozí užití

Oxidoreductases Acting on CH-CH Group Donors (2004-2005); Oxidoreductases (1983-2003); Acyl Coenzyme A (1980-1982); Coenzyme A (1973-1980); Valerates (1973-1982)

Historická pozn.

2006(1973)

Veřejná pozn.

2006; ISOVALERYL-COA DEHYDROGENASE was indexed under OXIDOREDUCTASES ACTING ON CH-CH GROUP DONORS 2004-2005, under OXIDOREDUCTASES 1983-2003, under ACYL COENZYME A 1980-1982, COENZYME A 1973-1980, and under VALERATES 1973-1982