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

Basic Information

Enzyme Number

1.3.99.13

Official Name

long-chain-acyl-CoA dehydrogenase

Name from literature

acyl coA dehydrogenase

Pathway from literature

long-chain fatty acid beta-oxidation

Pathway from KEGG

Lipid Metabolism; Fatty acid metabolism; map00071

Cellular Processes; Endocrine System; PPAR signaling pathway; map03320

Organisms

Human (9606)

Genome localization

2q34-q35[33 ],

Comments

A flavoprotein (FAD); forms with another flavoprotein ('electron-transferring flavoprotein') and EC 1.5.5.1 a system reducing ubiquinone and other acceptors. Not identical with EC 1.3.99.2 (butyryl-CoA dehydrogenase), EC 1.3.99.3 (acyl-CoA dehydrogenase), EC 1.3.99.10 (isovaleryl-CoA dehydrogenase) or EC 1.3.99.12 (2-methylacyl-CoA dehydrogenase).

Rate-limiting Description

"Although the rate-limiting step in the inactivation of the other acyl-CoA dehydrogenases can involve the abstraction of a proton at C-4, this is not the case with glutaryl-CoA dehydrogenase." (12716879)

Regulatory Information

Upstream transcription factor

4688

Regulatory type

Detail

transcriptional factor;activator protein 2(4688)

"Characterization of the human very-long-chain acyl-CoA dehydrogenase gene promoter region: a role for activator protein 2.#Characterization of the bidirectional promoter region between the human genes encoding VLCAD and PSD-95." (10607478#14611808#11108740)

key enzyme;

"Very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) is a key enzyme catalysing the dehydrogenation of long-chain fatty acids in mitochondrial beta-oxidation." (16880373#10832093)

transcriptional factor;

"We conclude that the developmental and tissue-specific regulation of the human LCAD gene is mediated, in part, by these nuclear hormone receptor transcription factors." (9003458)

Gene ontology

Gene ontology

GO:0050660 (F) FAD binding [P28330 ];
GO:0006631 (P) fatty acid metabolic process [P28330 ];
GO:0009055 (F) electron carrier activity [P28330 ];
GO:0005759 (C) mitochondrial matrix [P28330 ];
GO:0055114 (P) oxidation reduction [P28330 ];
GO:0004466 (F) long-chain-acyl-CoA dehydrogenase activity [P28330 ];

Subcellular localization

Localization

mitochondrion [P28330 ];

Disease relevance

Disease

Defects in ACADL are the cause of long-chain acyl-CoA dehydrogenase deficiency (LCAD deficiency) [MIM:201460]. LCAD deficiency leads to non-ketotic hypoglycemia [P28330 ];

Links

SwissProt

P28330

Entrez Gene

33

HPRD

01939



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  Last Modified: 2009-03-24  
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