Omega-hydroxyceramide transacylase involved in the synthesis of omega-O-acylceramides (esterified omega-hydroxyacyl-sphingosine; EOS), which are extremely hydrophobic lipids involved in skin barrier formation (PubMed:27751867, PubMed:28248318). Catalyzes the last step of the synthesis of omega-O-acylceramides by transferring linoleic acid from triglycerides to an omega-hydroxyceramide (PubMed:27751867, PubMed:28248318). Omega-O-acylceramides, are required for the biogenesis of lipid lamellae in the stratum corneum and the formation of the cornified lipid envelope which are essential for the epidermis barrier function (PubMed:22246504, PubMed:27751867, PubMed:28248318). These lipids also play a role in keratinocyte differentiation (By similarity). May also act on omega-hydroxylated ultra-long chain fatty acids (omega-OH ULCFA) and acylglucosylceramides (GlcEOS) (By similarity). a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + an N-(omega-hydroxy-ultra-long chain fatty acyl)-sphingoid base = a diacylglycerol + an N-[omega-(9Z,12Z-octadecadienoyloxy)-O-ultra-long chain fatty acyl]-sphingoid base a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + an N-(omega-hydroxy-ultra-long chain fatty acyl)-sphing-4-enine = a diacylglycerol + an N-(omega-(9Z,12Z-octadecadienoyloxy)-ultra-long chain fatty acyl)-sphing-4-enine 1,2,3-tri-(9Z,12Z)-octadecadienoylglycerol + N-(30-hydroxytriacontanoyl)-sphing-4-enine = di-(9Z,12Z)-octadecadienoylglycerol + N-[30-(9Z,12Z-octadecadienoyloxy)-triacontanoyl]-sphing-4-enine a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + N-(28-hydroxyoctacosanoyl)-sphing-4-enine = a diacylglycerol + N-(28-(9Z,12Z-octadecadienoyloxy)-octacosanoyl)-sphing-4-enine a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + N-(32-hydroxydotriacontanoyl)-sphing-4-enine = a diacylglycerol + N-(32-(9Z,12Z-octadecadienoyloxy)-dotricontanoyl)-sphing-4-enine a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + N-(32-hydroxydotriacontenoyl)-sphing-4-enine = a diacylglycerol + an N-(32-(9Z,12Z-octadecadienoyloxy)-dotriacontenoyl)-sphing-4-enine a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + an N-(34-hydroxytetratriacontenoyl)-sphing-4-enine = a diacylglycerol + an N-(34-(9Z,12Z-octadecadienoyloxy)-tetratriacontenoyl)-sphing-4-enine a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + an N-(34-hydroxytetratriacontadienoyl)-sphing-4-enine = a diacylglycerol + an N-(34-(9Z,12Z-octadecadienoyloxy)-tetratriacontadienoyl)-sphing-4-enine a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + an N-(36-hydroxyhexatriacontenoyl)-sphing-4-enine = a diacylglycerol + an N-(36-(9Z,12Z-octadecadienoyloxy)-hexatriacontenoyl)-sphing-4-enine a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + an N-(36-hydroxyhexatriacontadienoyl)-sphing-4-enine = a diacylglycerol + an N-(36-(9Z,12Z-octadecadienoyloxy)-hexatriacontadienoyl)-sphing-4-enine a (9Z,12Z)-octadecadienoyl-containing triacyl-sn-glycerol + an N-(38-hydroxyoctatriacontenoyl)-sphing-4-enine = a diacylglycerol + an N-(38-(9Z,12Z-octadecadienoyloxy)-octatriacontenoyl)-sphing-4-enine Expressed in the digestive system. Expressed in the epidermis of skin keratinocytes. Strongly expressed in the granular layer. Expressed in the upper epidermis and eccrine sweat glands of the dermis and in the region of keratin filament bundles, which is more pronounced in upper epidermal layers and in the lower cornified layers. Up-regulated upon induced differentiation of keratinocytes. The disease is caused by variants affecting the gene represented in this entry. Inactive. Inactive.