ACADSB 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. Belongs to the acyl-CoA dehydrogenase family. Ubiquitous. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC; Lipid Metabolism - fatty acid; Mitochondrial; Oxidoreductase
Chromosomal Location of human Ortholog: 10q26.13
Cellular Component:  mitochondrial matrix; mitochondrion
Molecular Function:  acyl-CoA dehydrogenase activity; flavin adenine dinucleotide binding
Biological Process:  branched-chain amino acid catabolic process; fatty acid metabolic process; oxidation-reduction process
Disease: 2-methylbutyryl-coa Dehydrogenase Deficiency
Reference #:  P45954 (UniProtKB)
Alt. Names/Synonyms: 2-MEBCAD; 2-methyl branched chain acyl-CoA dehydrogenase; 2-methylbutyryl-CoA dehydrogenase; 2-methylbutyryl-coenzyme A dehydrogenase; ACAD7; ACADSB; ACDSB; acyl-CoA dehydrogenase short/branched chain; acyl-Coenzyme A dehydrogenase, short/branched chain; SBCAD; Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
Gene Symbols: ACADSB
Molecular weight: 47,485 Da
Basal Isoelectric point: 6.53  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  AlphaFold  |  STRING  |  cBioPortal  |  Wikipedia  |  Reactome  |  neXtProt  |  Protein Atlas  |  BioGPS  |  Pfam  |  RCSB PDB  |  ENZYME  |  Phospho3D  |  Phospho.ELM  |  NetworKIN  |  GeneCards  |  UniProtKB  |  Entrez-Gene  |  GenPept  |  Ensembl Gene