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Details on Person DEGS1 (DES1, sphingolipid delta(4)-desaturase 1 / “degenerat...
| Class:Id | Summation:428244 |
|---|---|
| _displayName | DEGS1 (DES1, sphingolipid delta(4)-desaturase 1 / “degenerat... |
| _timestamp | 2023-10-07 08:03:28 |
| created | [InstanceEdit:428277] D'Eustachio, P, 2009-07-03 |
| modified | [InstanceEdit:428280] D'Eustachio, P, 2009-07-03 [InstanceEdit:9843169] Stephan, Ralf, 2023-09-01 [InstanceEdit:9845639] Stephan, Ralf, 2023-10-07 |
| text | DEGS1 (DES1, sphingolipid delta(4)-desaturase 1 / “degenerative spermatocyte homolog 1”) enzyme associated with the cytosolic face of the endoplasmic reticulum catalyzes the desaturation of dihydroceramide to form ceramide (Cadena et al. 1997; Ternes et al. 2002). The stoichiometry and cofactor requirements of the reaction are inferred from those observed in studies of ceramide synthesis in vitro catalyzed by rat liver microsomes (Michel et al. 1997). Apparently, DEGS1 is part of an enzyme complex, together with non-heme cytochrome b5 carrying Fe2+, that provides the reduction equivalent, which is subsequently refreshed by NADH (Geeraert et al., 1997; reviewed by Fabrias et al., 2011). The reaction is necessary as a step in ceramide biosynthesis, dihydroceramides and their downstream products like dihydrosphingomyelins are biologically active, for example, as part of lipid rafts, and inhibition of DEGS1 is a therapeutic target in cancer treatment (reviewed by Fabrias et al., 2011; Tzou et al., 2023). Mutations in DEGS1 cause hypomyelinating leukodystrophy (HLD18, MIM:618404) (reviewed by Wong et al., 2023). |
| (summation) | [Reaction:428259] DEGS1 dehydrogenates dihydroceramide [Homo sapiens] |
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No pathways have been reviewed or authored by DEGS1 (DES1, sphingolipid delta(4)-desaturase 1 / “degenerat... (428244)
