A gene on chromosome 15q14-q15 that encodes isovaleryl-CoA dehydrogenase, a mitochondrial matrix enzyme that catalyses the third step in leucine catabolism. Defects in IVD leads to a toxic accumulation of isovaleric acid in the brain or isovaleric acidaemia. Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved. Pert. to a diagnosis.
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