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Details on Person UniProt:Q9GZR5 ELOVL4
| Class:Id | ReferenceGeneProduct:54270 |
|---|---|
| _chainChangeLog | chain:1-314 added on Fri February 6 2015 |
| _displayName | UniProt:Q9GZR5 ELOVL4 |
| _timestamp | 2025-05-21 21:48:51 |
| chain | chain:1-314 |
| checksum | B2EBCE54D868E96E |
| comment | FUNCTION Catalyzes the first and rate-limiting reaction of the four reactions that constitute the long-chain fatty acids elongation cycle. This endoplasmic reticulum-bound enzymatic process allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids (VLCFAs) per cycle. Condensing enzyme that catalyzes the synthesis of very long chain saturated (VLC-SFA) and polyunsaturated (PUFA) fatty acids that are involved in multiple biological processes as precursors of membrane lipids and lipid mediators. May play a critical role in early brain and skin development.CATALYTIC ACTIVITY a very-long-chain acyl-CoA + malonyl-CoA + H(+) = a very-long-chain 3-oxoacyl-CoA + CO2 + CoACATALYTIC ACTIVITY tetracosanoyl-CoA + malonyl-CoA + H(+) = 3-oxohexacosanoyl-CoA + CO2 + CoACATALYTIC ACTIVITY hexacosanoyl-CoA + malonyl-CoA + H(+) = 3-oxooctacosanyol-CoA + CO2 + CoACATALYTIC ACTIVITY octacosanoyl-CoA + malonyl-CoA + H(+) = 3-oxo-triacontanoyl-CoA + CO2 + CoACATALYTIC ACTIVITY triacontanoyl-CoA + malonyl-CoA + H(+) = 3-oxo-dotriacontanoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (19Z,22Z,25Z,28Z,31Z)-tetratriacontapentaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(21Z,24Z,27Z,30Z,33Z)-hexatriacontapentaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (7Z,10Z,13Z,16Z)-docosatetraenoyl-CoA + malonyl-CoA + H(+) = (9Z,12Z,15Z,18Z)-3-oxotetracosatetraenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (11Z,14Z,17Z,20Z,23Z)-hexacosapentaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(13Z,16Z,19Z,22Z,25Z)-octacosapentaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (13Z,16Z,19Z,22Z,25Z)-octacosapentaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(15Z,18Z,21Z,24Z,27Z)-triacontapentaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (15Z,18Z,21Z,24Z,27Z)-triacontapentaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(17Z,20Z,23Z,26Z,29Z)-dotriacontapentaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (17Z,20Z,23Z,26Z,29Z)-dotriacontapentaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(19Z,22Z,25Z,28Z,31Z)-tetratriacontapentaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (21Z,24Z,27Z,30Z,33Z)-hexatriacontapentaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(23Z,26Z,29Z,32Z,35Z)-octatriacontapentaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (11Z,14Z,17Z,20Z)-hexacosatetraenoyl-CoA + malonyl-CoA + H(+) = (13Z,16Z,19Z,22Z)-3-oxooctacosatetraenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (13Z,16Z,19Z,22Z)-octacosatetraenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(15Z,18Z,21Z,24Z)-triacontatetraenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (15Z,18Z,21Z,24Z)-triacontatetraenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(17Z,20Z,23Z,26Z)-dotriacontatetraenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (17Z,20Z,23Z,26Z)-dotriacontatetraenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(19Z,22Z,25Z,28Z)-tetratriacontatetraenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (19Z,22Z,25Z,28Z)-tetratriacontatetraenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(21Z,24Z,27Z,30Z)-hexatriacontatetraenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (21Z,24Z,27Z,30Z)-hexatriacontatetraenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(23Z,26Z,29Z,32Z)-octatriacontatetraenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(8Z,11Z,14Z,17Z,20Z,23Z)-hexacosahexaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (8Z,11Z,14Z,17Z,20Z,23Z)-hexacosahexaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(10Z,13Z,16Z,19Z,22Z,25Z)-octacosahexaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (10Z,13Z,16Z,19Z,22Z,25Z)-octacosahexaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(12Z,15Z,18Z,21Z,24Z,27Z)-triacontahexaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (12Z,15Z,18Z,21Z,24Z,27Z)-triacontahexaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(14Z,17Z,20Z,23Z,26Z,29Z)-dotriacontahexaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (14Z,17Z,20Z,23Z,26Z,29Z)-dotriacontahexaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(16Z,19Z,22Z,25Z,28Z,31Z)-tetratriacontahexaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (16Z,19Z,22Z,25Z,28Z,31Z)-tetratriacontahexaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(18Z,21Z,24Z,27Z,30Z,33Z)-hexatriacontahexaenoyl-CoA + CO2 + CoACATALYTIC ACTIVITY (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoA + malonyl-CoA + H(+) = 3-oxo-(11Z,14Z,17Z,20Z,23Z)-hexacosapentaenoyl-CoA + CO2 + CoAPATHWAY Lipid metabolism; fatty acid biosynthesis.SUBUNIT Oligomer.INTERACTION Expressed in the retina and at much lower level in the brain. Ubiquitous, highest expression in thymus, followed by testis, small intestine, ovary, and prostate. Little or no expression in heart, lung, liver, or leukocates.DOMAIN The C-terminal di-lysine motif confers endoplasmic reticulum localization.PTM N-glycosylated.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the ELO family. ELOVL4 subfamily. |
| description | recommendedName: fullName evidence="2"Very long chain fatty acid elongase 4 ecNumber evidence="2 8 10"2.3.1.199 alternativeName: fullName evidence="2"3-keto acyl-CoA synthase ELOVL4 alternativeName: fullName evidence="2"ELOVL fatty acid elongase 4 shortName evidence="2"ELOVL FA elongase 4 alternativeName: fullName evidence="2"Elongation of very long chain fatty acids protein 4 alternativeName: fullName evidence="2"Very long chain 3-ketoacyl-CoA synthase 4 alternativeName: fullName evidence="2"Very long chain 3-oxoacyl-CoA synthase 4 |
| geneName | ELOVL4 |
| identifier | Q9GZR5 |
| isSequenceChanged | FALSE |
| keyword | Disease variant Endoplasmic reticulum Fatty acid biosynthesis Fatty acid metabolism Glycoprotein Ichthyosis Intellectual disability Lipid biosynthesis Lipid metabolism Membrane Neurodegeneration Proteomics identification Reference proteome Spinocerebellar ataxia Stargardt disease Transferase Transmembrane Transmembrane helix |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9862192] Weiser, Joel, 2024-02-26 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9948485] Weiser, Joel, 2025-05-21 |
| name | ELOVL4 |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:9000565] ENSEMBL:ENSG00000118402 ELOVL4 [Homo sapiens] |
| secondaryIdentifier | ELOV4_HUMAN B2R6B5 Q5TCS2 Q86YJ1 Q9H139 |
| sequenceLength | 314 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:548705] ELOVL4 [endoplasmic reticulum membrane] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:Q9GZR5 ELOVL4 (54270)
