| Entry |
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| Name |
very-long-chain acyl-CoA dehydrogenase;
ACADVL (gene name).
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| Class |
Oxidoreductases;
Acting on the CH-CH group of donors;
With a flavin as acceptor
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| Sysname |
very-long-chain acyl-CoA:electron-transfer flavoprotein 2,3-oxidoreductase
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| Reaction(IUBMB) |
a very-long-chain acyl-CoA + electron-transfer flavoprotein = a very-long-chain trans-2,3-dehydroacyl-CoA + reduced electron-transfer flavoprotein
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| Reaction(KEGG) |
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| Substrate |
very-long-chain acyl-CoA [CPD: C20876];
electron-transfer flavoprotein [CPD: C04253]
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| Product |
very-long-chain trans-2,3-dehydroacyl-CoA [CPD: C20879];
reduced electron-transfer flavoprotein [CPD: C04570]
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| Comment |
Contains FAD as prosthetic group. One of several enzymes that catalyse the first step in fatty acids beta-oxidation. The enzyme is most active toward long-chain acyl-CoAs such as C14, C16 and C18, but is also active with very-long-chain acyl-CoAs up to 24 carbons. It shows no activity for substrates of less than 12 carbons. Its specific activity towards palmitoyl-CoA is more than 10-fold that of the long-chain acyl-CoA dehydrogenase [1]. cf. EC 1.3.8.1, short-chain acyl-CoA dehydrogenase, EC 1.3.8.7, medium-chain acyl-CoA dehydrogenase, and EC 1.3.8.8, long-chain acyl-CoA dehydrogenase.
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| History |
EC 1.3.8.9 created 1961 as EC 1.3.2.2, transferred 1964 to EC 1.3.99.3, part transferred 2012 to EC 1.3.8.9
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| Pathway |
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| Orthology |
| K09479 | very long chain acyl-CoA dehydrogenase |
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| Genes |
» show all
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| Reference |
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| Authors |
Izai K, Uchida Y, Orii T, Yamamoto S, Hashimoto T |
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| Title |
Novel fatty acid beta-oxidation enzymes in rat liver mitochondria. I. Purification and properties of very-long-chain acyl-coenzyme A dehydrogenase. |
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| Journal |
J Biol Chem 267:1027-33 (1992) |
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| Sequence |
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| Reference |
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| Authors |
Aoyama T, Souri M, Ushikubo S, Kamijo T, Yamaguchi S, Kelley RI, Rhead WJ, Uetake K, Tanaka K, Hashimoto T |
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| Title |
Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients. |
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| Journal |
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| Sequence |
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| Reference |
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| Authors |
McAndrew RP, Wang Y, Mohsen AW, He M, Vockley J, Kim JJ |
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| Title |
Structural basis for substrate fatty acyl chain specificity: crystal structure of human very-long-chain acyl-CoA dehydrogenase. |
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| Journal |
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| Sequence |
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| Other DBs |
| ExplorEnz - The Enzyme Database: | 1.3.8.9 |
| ExPASy - ENZYME nomenclature database: | 1.3.8.9 |
| BRENDA, the Enzyme Database: | 1.3.8.9 |
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