We use cookies to enhance the usability of our website. If you continue, we'll assume that you are happy to receive all cookies. More information. Don't show this again.
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 encoding different isoforms have been found for this gene. [provided by RefSeq, Apr 2009]
Enzymes ENZYME proteins Oxidoreductases SPOCTOPUS predicted membrane proteins Predicted intracellular proteins Disease related genes Potential drug targets Protein evidence (Kim et al 2014) Protein evidence (Ezkurdia et al 2014)
Show all
GO:0000062 [fatty-acyl-CoA binding] GO:0003995 [acyl-CoA dehydrogenase activity] GO:0005654 [nucleoplasm] GO:0005739 [mitochondrion] GO:0005759 [mitochondrial matrix] GO:0006552 [leucine catabolic process] GO:0008152 [metabolic process] GO:0008470 [isovaleryl-CoA dehydrogenase activity] GO:0009055 [electron carrier activity] GO:0009083 [branched-chain amino acid catabolic process] GO:0016627 [oxidoreductase activity, acting on the CH-CH group of donors] GO:0033539 [fatty acid beta-oxidation using acyl-CoA dehydrogenase] GO:0034641 [cellular nitrogen compound metabolic process] GO:0044281 [small molecule metabolic process] GO:0050660 [flavin adenine dinucleotide binding] GO:0052890 [oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor] GO:0055088 [lipid homeostasis] GO:0055114 [oxidation-reduction process]
