This gene encodes a member of the acyl-CoA dehydrogenase family of enzymes that catalyze the dehydrogenation of acyl-CoA derivatives in the metabolism of fatty acids or branch chained amino acids. The encoded protein is a mitochondrial enzyme that functions in catabolism of the branched-chain amino acid valine. Defects in this gene are the cause of isobutyryl-CoA dehydrogenase deficiency.
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Protein Aliases: ACAD 8; ACAD-8; ACAD8; Activator-recruited cofactor 42 kDa component; Acyl-CoA dehydrogenase family member 8; acyl-CoA dehydrogenase family, member 8; acyl-Coenzyme A dehydrogenase family, member 8; ARC42; IBD; IBDH; Isobutyryl-CoA dehydrogenase, mitochondrial
Gene Aliases: 2310016C19Rik; ACAD-8; ACAD8; AI786953; ARC42; IBD; RGD1564209