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Details on Person Lactosylceramide (LacCer) is the central intermediate in gly...
| Class:Id | Summation:1606313 |
|---|---|
| _displayName | Lactosylceramide (LacCer) is the central intermediate in gly... |
| _timestamp | 2023-08-16 13:27:40 |
| created | [InstanceEdit:1606314] Jassal, Bijay, 2011-09-21 |
| modified | [InstanceEdit:9840967] Stephan, Ralf, 2023-08-03 [InstanceEdit:9842002] Stephan, Ralf, 2023-08-16 |
| text | Lactosylceramide (LacCer) is the central intermediate in glycosphingolipid catabolism. Beta-galactosidase (GLB1) cleaves the galactose moiety from LacCer to form glucosylceramide. LacCer accumulates in GLB1 deficiencies (MIM: 611458) like GM1-gangliosidosis but also in prosaposin deficiency (PSAPD, MIM: 611721), which shows saposin cofactors are essential for the reaction (Asp et al. 1969; Bradova et al., 1993). We follow Sandhoff & Sandhoff (2018), who state that Saposins B/C bind to LacCer. |
| (summation) | [Reaction:1606312] GLB1 hydrolyzes SapB/C:LacCer [Homo sapiens] |
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No pathways have been reviewed or authored by Lactosylceramide (LacCer) is the central intermediate in gly... (1606313)
