Reaction: isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2
- in pathway: Branched-chain amino acid catabolism
Mitochondrial isovaleryl dehydrogenase (IVD) catalyzes the reaction of isovaleryl-CoA and FAD to form beta-methylcrotonyl-CoA and FADH2 (Finocchiaro et al. 1978; Rhead and Tanaka 1980). Crystallographic studies demonstrated the existene of a tetramer of IVD polypeptides lacking an aminoterminal mitochondrial targeting sequence (Tiffany et al. 1997).
Reaction - small molecule participants:
bMC-CoA [mitochondrial matrix]
FADH2 [mitochondrial matrix]
FAD [mitochondrial matrix]
ISV-CoA [mitochondrial matrix]
Reactome.org reaction link: R-HSA-70745
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Reaction input - small molecules:
FAD(3-)
isovaleryl-CoA(4-)
Reaction output - small molecules:
3-methylbut-2-enoyl-CoA(4-)
FADH2(2-)
Reactome.org link: R-HSA-70745