Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

Official Name

butyryl-CoA dehydrogenase

Name from literature

medium chain acyl coA dehydrogenase

Pathway from literature

medium-chain fatty acid beta-oxidation

Pathway from KEGG

Lipid Metabolism; Fatty acid metabolism; map00071

Amino Acid Metabolism; Valine, leucine and isoleucine degradation; map00280

Carbohydrate Metabolism; Butanoate metabolism; map00650


Human (9606)

Genome localization

12q22-qter[35 ],


A flavoprotein; forms, with another flavoprotein ('electron-transferring flavoproteins') and EC electron-transferring-flavoprotein dehydrogenase, a system reducing ubiquinone and other acceptors.

Rate-limiting Description

"In contrast, the activity and steady-state levels of medium-chain acyl-CoA dehydrogenase, which catalyzes a rate-limiting step in FAO, were not significantly reduced until the HF stage, indicating additional control at the translational or post-translational levels in the hypertrophied but nonfailing ventricle." (8941110)

"Results of RV pressure overload studies in mice transgenic for the promoter region of the gene encoding human medium-chain acyl-CoA dehydrogenase (MCAD, which catalyzes a rate-limiting step in the FAO cycle) fused to a chloramphenicol acetyltransferase reporter confirmed that repression of MCAD gene expression in the hypertrophied ventricle occurred at the transcriptional level." (9177236)

Regulatory Information

Regulatory type



"Lastly, competitive inhibition studies using the ETF fluorescence reduction assay suggested that SCAD E368G and IVD E254G do not effectively compete with the wild-type enzymes for the physiological electron acceptor ETF." (16376132)

Gene ontology

Gene ontology

GO:0050660 (F) FAD binding [P16219 ];
GO:0009055 (F) electron carrier activity [P16219 ];
GO:0006635 (P) fatty acid beta-oxidation [P16219 ];
GO:0005759 (C) mitochondrial matrix [P16219 ];
GO:0055114 (P) oxidation reduction [P16219 ];
GO:0004085 (F) butyryl-CoA dehydrogenase activity [P16219 ];

Subcellular localization


mitochondrion [P16219 ];

Disease relevance


Defects in ACADS are the cause of short-chain acyl-CoA dehydrogenase deficiency (SCAD deficiency) [MIM:201470]. It is an autosomal recessive disorder resulting in acute acidosis and muscle weakness in infants, and a form of lipid-storage myopathy in adults [P16219 ];




Entrez Gene




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  Last Modified: 2009-03-24  
  Design by Zhao Min