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. a very-long-chain acyl-CoA + H(+) + malonyl-CoA = a very-long-chain 3-oxoacyl-CoA + CO2 + CoA H(+) + malonyl-CoA + tetracosanoyl-CoA = 3-oxohexacosanoyl-CoA + CO2 + CoA H(+) + hexacosanoyl-CoA + malonyl-CoA = 3-oxooctacosanyol-CoA + CO2 + CoA H(+) + malonyl-CoA + octacosanoyl-CoA = 3-oxo-triacontanoyl-CoA + CO2 + CoA H(+) + malonyl-CoA + triacontanoyl-CoA = 3-oxo-dotriacontanoyl-CoA + CO2 + CoA (19Z,22Z,25Z,28Z,31Z)-tetratriacontapentaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(21Z,24Z,27Z,30Z,33Z)-hexatriacontapentaenoyl-CoA + CO2 + CoA (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoA + CO2 + CoA (7Z,10Z,13Z,16Z)-docosatetraenoyl-CoA + H(+) + malonyl-CoA = (9Z,12Z,15Z,18Z)-3-oxotetracosatetraenoyl-CoA + CO2 + CoA (11Z,14Z,17Z,20Z,23Z)-hexacosapentaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(13Z,16Z,19Z,22Z,25Z)-octacosapentaenoyl-CoA + CO2 + CoA (13Z,16Z,19Z,22Z,25Z)-octacosapentaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(15Z,18Z,21Z,24Z,27Z)-triacontapentaenoyl-CoA + CO2 + CoA (15Z,18Z,21Z,24Z,27Z)-triacontapentaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(17Z,20Z,23Z,26Z,29Z)-dotriacontapentaenoyl-CoA + CO2 + CoA (17Z,20Z,23Z,26Z,29Z)-dotriacontapentaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(19Z,22Z,25Z,28Z,31Z)-tetratriacontapentaenoyl-CoA + CO2 + CoA (21Z,24Z,27Z,30Z,33Z)-hexatriacontapentaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(23Z,26Z,29Z,32Z,35Z)-octatriacontapentaenoyl-CoA + CO2 + CoA (11Z,14Z,17Z,20Z)-hexacosatetraenoyl-CoA + H(+) + malonyl-CoA = (13Z,16Z,19Z,22Z)-3-oxooctacosatetraenoyl-CoA + CO2 + CoA (13Z,16Z,19Z,22Z)-octacosatetraenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(15Z,18Z,21Z,24Z)-triacontatetraenoyl-CoA + CO2 + CoA (15Z,18Z,21Z,24Z)-triacontatetraenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(17Z,20Z,23Z,26Z)-dotriacontatetraenoyl-CoA + CO2 + CoA (17Z,20Z,23Z,26Z)-dotriacontatetraenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(19Z,22Z,25Z,28Z)-tetratriacontatetraenoyl-CoA + CO2 + CoA (19Z,22Z,25Z,28Z)-tetratriacontatetraenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(21Z,24Z,27Z,30Z)-hexatriacontatetraenoyl-CoA + CO2 + CoA (21Z,24Z,27Z,30Z)-hexatriacontatetraenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(23Z,26Z,29Z,32Z)-octatriacontatetraenoyl-CoA + CO2 + CoA (6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(8Z,11Z,14Z,17Z,20Z,23Z)-hexacosahexaenoyl-CoA + CO2 + CoA (8Z,11Z,14Z,17Z,20Z,23Z)-hexacosahexaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(10Z,13Z,16Z,19Z,22Z,25Z)-octacosahexaenoyl-CoA + CO2 + CoA (10Z,13Z,16Z,19Z,22Z,25Z)-octacosahexaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(12Z,15Z,18Z,21Z,24Z,27Z)-triacontahexaenoyl-CoA + CO2 + CoA (12Z,15Z,18Z,21Z,24Z,27Z)-triacontahexaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(14Z,17Z,20Z,23Z,26Z,29Z)-dotriacontahexaenoyl-CoA + CO2 + CoA (14Z,17Z,20Z,23Z,26Z,29Z)-dotriacontahexaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(16Z,19Z,22Z,25Z,28Z,31Z)-tetratriacontahexaenoyl-CoA + CO2 + CoA (16Z,19Z,22Z,25Z,28Z,31Z)-tetratriacontahexaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(18Z,21Z,24Z,27Z,30Z,33Z)-hexatriacontahexaenoyl-CoA + CO2 + CoA (9Z,12Z,15Z,18Z,21Z)-tetracosapentaenoyl-CoA + H(+) + malonyl-CoA = 3-oxo-(11Z,14Z,17Z,20Z,23Z)-hexacosapentaenoyl-CoA + CO2 + CoA Lipid metabolism; fatty acid biosynthesis. Oligomer. 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. The C-terminal di-lysine motif confers endoplasmic reticulum localization. N-glycosylated. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. Belongs to the ELO family. ELOVL4 subfamily. Retina International's Scientific Newsletter