Aliases for ACAD8 Gene
External Ids for ACAD8 Gene
Previous GeneCards Identifiers for ACAD8 Gene
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.[provided by RefSeq, Nov 2009]
GeneCards Summary for ACAD8 Gene
ACAD8 (Acyl-CoA Dehydrogenase Family Member 8) is a Protein Coding gene. Diseases associated with ACAD8 include Isobutyryl-Coa Dehydrogenase Deficiency and Acyl-Coa Dehydrogenase, Short-Chain, Deficiency Of. Among its related pathways are Valine, leucine and isoleucine degradation and Viral mRNA Translation. Gene Ontology (GO) annotations related to this gene include flavin adenine dinucleotide binding and acyl-CoA dehydrogenase activity. An important paralog of this gene is ACADS.
UniProtKB/Swiss-Prot Summary for ACAD8 Gene
Isobutyryl-CoA dehydrogenase which catalyzes one of the steps of the valine catabolic pathway (PubMed:11013134, PubMed:12359132, PubMed:16857760). To a lesser extent, is also able to catalyze the oxidation of (2S)-2-methylbutanoyl-CoA (PubMed:11013134, PubMed:12359132).