| Entry |
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| Name |
Acylglucosylceramide synthesis
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| Definition |
omega-OH-ULCFA-CoA+Sphinganine -- CERS3 -> omega-OH-Cer -- ABHD5+PNPLA1 -> Acyl-Cer -- UGCG -> Acyl-GlcCer |
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| Expanded |
C23027+C00836 -- 204219 -> C23028 -- 51099+285848 -> C23029 -- 7357 -> C23030 |
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| Class |
nt06545 Cornified envelope formation
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| Type |
Reference
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| Pathway |
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| Disease |
| H00734 Autosomal recessive congenital ichthyosis | |
| H00736 Dorfman-Chanarin syndrome | |
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| Gene |
| 204219 | CERS3; ceramide synthase 3 |
| 51099 | ABHD5; abhydrolase domain containing 5, lysophosphatidic acid acyltransferase |
| 285848 | PNPLA1; patatin like domain 1, omega-hydroxyceramide transacylase |
| 7357 | UGCG; UDP-glucose ceramide glucosyltransferase |
|
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| Metabolite |
| C23027 | Ultra-long-chain omega-hydroxy fatty acyl-CoA |
| C23028 | Ultra-long-chain omega-hydroxyceramide |
| C23029 | linoleate-esterified acylceramide |
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| Reference |
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| Authors |
Breiden B, Sandhoff K |
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| Title |
The role of sphingolipid metabolism in cutaneous permeability barrier formation. |
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| Journal |
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| Reference |
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| Authors |
Kien B, Grond S, Haemmerle G, Lass A, Eichmann TO, Radner FPW |
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| Title |
ABHD5 stimulates PNPLA1-mediated omega-O-acylceramide biosynthesis essential for a functional skin permeability barrier. |
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| Journal |
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| Reference |
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| Authors |
Ge F, Sun K, Hu Z, Dong X |
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| Title |
Role of Omega-Hydroxy Ceramides in Epidermis: Biosynthesis, Barrier Integrity and Analyzing Method. |
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| Journal |
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| LinkDB |
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