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Lyso-glycosphingolipids: presence and consequences.

Authors :
van Eijk M
Ferraz MJ
Boot RG
Aerts JMFG
Source :
Essays in biochemistry [Essays Biochem] 2020 Sep 23; Vol. 64 (3), pp. 565-578.
Publication Year :
2020

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.<br /> (© 2020 The Author(s).)

Details

Language :
English
ISSN :
1744-1358
Volume :
64
Issue :
3
Database :
MEDLINE
Journal :
Essays in biochemistry
Publication Type :
Academic Journal
Accession number :
32808655
Full Text :
https://doi.org/10.1042/EBC20190090