Names & Taxonomy
- Uniprot ID:
- P45954
- Entry Name:
- ACDSB_HUMAN
- Status:
- reviewed
- Protein Names:
- Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial (SBCAD) (EC 1.3.8.5) (2-methyl branched chain acyl-CoA dehydrogenase) (2-MEBCAD) (2-methylbutyryl-coenzyme A dehydrogenase) (2-methylbutyryl-CoA dehydrogenase)
- Gene Names:
- ACADSB
- Gene Names Primary:
- ACADSB
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 432
- Sequence:
- MEGLAVRLLRGSRLLRRNFLTCLSSWKIPPHVSKSSQSEALLNITNNGIHFAPLQTFTDEEMMIKSSVKKFAQEQIAPLVSTMDENSKMEKSVIQGLFQQGLMGIEVDPEYGGTGASFLSTVLVIEELAKVDASVAVFCEIQNTLINTLIRKHGTEEQKATYLPQLTTEKVGSFCLSEAGAGSDSFALKTRADKEGDYYVLNGSKMWISSAEHAGLFLVMANVDPTIGYKGITSFLVDRDTPGLHIGKPENKLGLRASSTCPLTFENVKVPEANILGQIGHGYKYAIGSLNEGRIGIAAQMLGLAQGCFDYTIPYIKERIQFGKRLFDFQGLQHQVAHVATQLEAARLLTYNAARLLEAGKPFIKEASMAKYYASEIAGQTTSKCIEWMGGVGYTKDYPVEKYFRDAKIGTIYEGASNIQLNTIAKHIDAEY
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Mitochondrion matrix.
Function
- Function:
- Has greatest activity toward short branched chain acyl-CoA derivative such as (s)-2-methylbutyryl-CoA, isobutyryl-CoA, and 2-methylhexanoyl-CoA as well as toward short straight chain acyl-CoAs such as butyryl-CoA and hexanoyl-CoA. Can use valproyl-CoA as substrate and may play a role in controlling the metabolic flux of valproic acid in the development of toxicity of this agent.
- Pathway:
- Lipid metabolism; mitochondrial fatty acid beta-oxidation.
- Catalytic Activity:
- Acyl-CoA + acceptor = 2,3-dehydroacyl-CoA + reduced acceptor.; 2-methylbutanoyl-CoA + electron-transfer flavoprotein = (E)-2-methylbut-2-enoyl-CoA + reduced electron-transfer flavoprotein + H(+).
- Cofactor:
- COFACTOR: Name=FAD; Xref=ChEBI:CHEBI:57692;
- Active Site:
- ACT_SITE 414 414 Proton acceptor.
- Cross Reference Drug Bank:
- DB00167 DB00313
- Gene Ontology Go:
- extracellular exosome
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
fatty acid metabolic process
lipid homeostasis
small molecule metabolic process - Gene Ontology Biological Process:
- branched-chain amino acid catabolic process
cellular nitrogen compound metabolic process
fatty acid beta-oxidation using acyl-CoA dehydrogenase
fatty acid metabolic process
lipid homeostasis
small molecule metabolic 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:
- extracellular exosome
mitochondrial matrix
mitochondrion - Keywords:
- 3D-structure
Acetylation
Alternative splicing
Complete proteome
Disease mutation
FAD
Fatty acid metabolism
Flavoprotein
Lipid metabolism
Mitochondrion
Oxidoreductase
Phosphoprotein
Polymorphism
Reference proteome
Transit peptide