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 Metabolism. 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).