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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.<\/jats:p>","DOI":"10.1042\/ebc20190090","type":"journal-article","created":{"date-parts":[[2020,8,18]],"date-time":"2020-08-18T11:15:41Z","timestamp":1597749341000},"page":"565-578","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":60,"title":["Lyso-glycosphingolipids: presence and consequences"],"prefix":"10.1042","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7751-4112","authenticated-orcid":false,"given":"Marco","family":"van Eijk","sequence":"additional","affiliation":[{"name":"Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria\u00a0J.","family":"Ferraz","sequence":"additional","affiliation":[{"name":"Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rolf\u00a0G.","family":"Boot","sequence":"additional","affiliation":[{"name":"Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Johannes\u00a0M.F.G.","family":"Aerts","sequence":"additional","affiliation":[{"name":"Leiden Institute of Chemistry, Leiden University, Einsteinweg 55, 2300 RA, Leiden, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2020,8,18]]},"reference":[{"key":"2021111610310634600_B1","volume-title":"A Treatise on the Chemical constitution of the brain","author":"Thudichum","year":"1884"},{"key":"2021111610310634600_B2","doi-asserted-by":"publisher","first-page":"6387","DOI":"10.1021\/cr2002917","article-title":"Sphingolipid and glycosphingolipid metabolic pathways in the era of sphingolipidomics","volume":"111","author":"Merrill","year":"2011","journal-title":"Chem. 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