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

Basic Information

Enzyme Number

1.3.99.2

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

Organisms

Human (9606)

Genome localization

12q22-qter[35 ],

Comments

A flavoprotein; forms, with another flavoprotein ('electron-transferring flavoproteins') and EC 1.5.5.1 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

Detail

inhibitor;

"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

Localization

mitochondrion [P16219 ];

Disease relevance

Disease

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 ];

Links

SwissProt

P16219

Entrez Gene

35

HPRD

06053



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  Last Modified: 2009-03-24  
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