Isovaleryl-CoA Dehydrogenase

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).

Year introduced: 2006(1973)

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Tree Number(s): D08.811.682.660.462

MeSH Unique ID: D050769

Registry Number: EC 1.3.8.4

Entry Terms:

• Dehydrogenase, Isovaleryl-CoA
• Isovaleryl CoA Dehydrogenase
• CoA Dehydrogenase, Isovaleryl
• Dehydrogenase, Isovaleryl CoA
• Isovaleryl-Coenzyme A Dehydrogenase
• Dehydrogenase, Isovaleryl-Coenzyme A
• Isovaleryl Coenzyme A Dehydrogenase

Previous Indexing:

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

All MeSH Categories
Chemicals and Drugs Category
Enzymes and Coenzymes
Enzymes
Oxidoreductases
Oxidoreductases Acting on CH-CH Group Donors
Isovaleryl-CoA Dehydrogenase