NP_055199.1
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NCBI GenBank Nucleotide #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
38,359 Da
NCBI Official Full Name
isobutyryl-CoA dehydrogenase, mitochondrial
NCBI Official Synonym Full Names
acyl-CoA dehydrogenase family member 8
NCBI Protein Information
isobutyryl-CoA dehydrogenase, mitochondrial
UniProt Protein Name
Isobutyryl-CoA dehydrogenase, mitochondrial
UniProt Synonym Protein Names
Activator-recruited cofactor 42 kDa component; ARC42; Acyl-CoA dehydrogenase family member 8; ACAD-8
UniProt Synonym Gene Names
UniProt Entry Name
ACAD8_HUMAN
NCBI Summary for ACAD8
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]
UniProt Comments for ACAD8
ACAD8: Has very high activity toward isobutyryl-CoA. Is an isobutyryl-CoA dehydrogenase that functions in valine catabolism. Plays a role in transcriptional coactivation within the ARC complex. Defects in ACAD8 are the cause of isobutyryl-CoA dehydrogenase deficiency (IBDD). The symptoms of IBDD generally appear until late in infancy or in childhood and can include poor feeding and growth (failure to thrive), a weakened and enlarged heart (dilated cardiomyopathy), seizures, and low numbers of red blood cells (anemia). Belongs to the acyl-CoA dehydrogenase family.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; Mitochondrial; EC 1.3.99.-; Oxidoreductase
Chromosomal Location of Human Ortholog: 11q25
Cellular Component: mitochondrial matrix; mitochondrion
Molecular Function: acyl-CoA binding; acyl-CoA dehydrogenase activity; electron carrier activity; FAD binding
Biological Process: branched chain family amino acid catabolic process; fatty acid beta-oxidation using acyl-CoA dehydrogenase; lipid homeostasis; lipid metabolic process; regulation of transcription, DNA-dependent; transcription, DNA-dependent; valine catabolic process
Disease: Isobutyryl-coa Dehydrogenase Deficiency
Research Articles on ACAD8
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Pathways associated with ACAD8 elisa kit
Diseases associated with ACAD8 elisa kit
Organs/Tissues associated with ACAD8 elisa kit
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