isovaleryl CoA dehydrogenase (IVD)

Function

• amino-acid degradation; L-leucine degradation
• HMG-CoA from 3-isovaleryl-CoA: step 1/3

3-methylbutanoyl-CoA + acceptor
     <-->
3-methylbut-2-enoyl-CoA + reduced acceptor

Cofactor: FAD

Structure

• homotetramer
• belongs to the acyl-CoA dehydrogenase family

Compartment

mitochondrial matrix

Pathology

• defects in IVD are the cause of isovaleric acidemia

Laboratory

• IVD gene mutation

More general terms

• dehydrogenase

Database

• UniProt: http://www.uniprot.org/uniprot/P26440.html
• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=3712
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:3712
• OMIM: https://mirror.omim.org/entry/243500
• OMIM: https://mirror.omim.org/entry/607036