Names & Taxonomy
- Uniprot ID:
- Q9UKU7
- Entry Name:
- ACAD8_HUMAN
- Status:
- reviewed
- Protein Names:
- Isobutyryl-CoA dehydrogenase, mitochondrial (EC 1.3.99.-) (Activator-recruited cofactor 42 kDa component) (ARC42) (Acyl-CoA dehydrogenase family member 8) (ACAD-8)
- Gene Names:
- ACAD8 ARC42 IBD
- Gene Names Primary:
- ACAD8
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 415
- Sequence:
- MLWSGCRRFGARLGCLPGGLRVLVQTGHRSLTSCIDPSMGLNEEQKEFQKVAFDFAAREMAPNMAEWDQKELFPVDVMRKAAQLGFGGVYIQTDVGGSGLSRLDTSVIFEALATGCTSTTAYISIHNMCAWMIDSFGNEEQRHKFCPPLCTMEKFASYCLTEPGSGSDAASLLTSAKKQGDHYILNGSKAFISGAGESDIYVVMCRTGGPGPKGISCIVVEKGTPGLSFGKKEKKVGWNSQPTRAVIFEDCAVPVANRIGSEGQGFLIAVRGLNGGRINIASCSLGAAHASVILTRDHLNVRKQFGEPLASNQYLQFTLADMATRLVAARLMVRNAAVALQEERKDAVALCSMAKLFATDECFAICNQALQMHGGYGYLKDYAVQQYVRDSRVHQILEGSNEVMRILISRSLLQE
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion
Function
- Function:
- 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.
- Pathway:
- Amino-acid degradation; L-valine degradation.
- Catalytic Activity:
- Isobutyryl-CoA + ETF = methylacrylyl-CoA + reduced ETF.
- Cofactor:
- COFACTOR: Name=FAD; Xref=ChEBI:CHEBI:57692;
- Active Site:
- ACT_SITE 398 398 Proton acceptor.
- Cross Reference Drug Bank:
- DB03147
- Gene Ontology Go:
- mitochondrial matrix
mitochondrion
acyl-CoA dehydrogenase activity
electron carrier activity
fatty-acyl-CoA binding
flavin adenine dinucleotide binding
oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor
branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
fatty acid beta-oxidation using acyl-CoA dehydrogenase
lipid homeostasis
lipid metabolic process
regulation of transcription, DNA-templated
small molecule metabolic process
transcription, DNA-templated
valine catabolic process - Gene Ontology Biological Process:
- branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
fatty acid beta-oxidation using acyl-CoA dehydrogenase
lipid homeostasis
lipid metabolic process
regulation of transcription, DNA-templated
small molecule metabolic process
transcription, DNA-templated
valine catabolic process - Gene Ontology Molecular Function:
- acyl-CoA dehydrogenase activity
electron carrier activity
fatty-acyl-CoA binding
flavin adenine dinucleotide binding
oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor - Gene Ontology Cellular Component:
- mitochondrial matrix
mitochondrion - Keywords:
- 3D-structure
Acetylation
Activator
Alternative splicing
Branched-chain amino acid catabolism
Complete proteome
Direct protein sequencing
Disease mutation
FAD
Flavoprotein
Mitochondrion
Oxidoreductase
Reference proteome
Transcription
Transcription regulation
Transit peptide