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- W3073842499 abstract "Abstract Lyso-glycosphingolipids are generated in excess in glycosphingolipid storage disorders. In the course of these pathologies glycosylated sphingolipid species accumulate within lysosomes due to flaws in the respective lipid degrading machinery. Deacylation of accumulating glycosphingolipids drives the formation of lyso-glycosphingolipids. In lysosomal storage diseases such as Gaucher Disease, Fabry Disease, Krabbe disease, GM1 -and GM2 gangliosidosis, Niemann Pick type C and Metachromatic leukodystrophy massive intra-lysosomal glycosphingolipid accumulation occurs. The lysosomal enzyme acid ceramidase generates the deacylated lyso-glycosphingolipid species. This review discusses how the various lyso-glycosphingolipids are synthesized, how they may contribute to abnormal immunity in glycosphingolipid storing lysosomal diseases and what therapeutic opportunities exist." @default.
- W3073842499 created "2020-08-24" @default.
- W3073842499 creator A5013935159 @default.
- W3073842499 creator A5040386068 @default.
- W3073842499 creator A5050568897 @default.
- W3073842499 creator A5088523142 @default.
- W3073842499 date "2020-08-18" @default.
- W3073842499 modified "2023-10-14" @default.
- W3073842499 title "Lyso-glycosphingolipids: presence and consequences" @default.
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