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ACAD8 is 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.
Gene Name: | acyl-CoA dehydrogenase family, member 8 |
Synonyms: | ACAD8, ARC42, ACAD-8, IBD |
Target Sequences: | NM_014384 NP_055199.1 Q9UKU7 |
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