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Altogether, they are called sphingolipids and are particularly abundant in neuronal plasma membranes, where, via interactions with the other membrane lipids and membrane proteins, they play a specific role in modulating the cell signaling processes. The metabolic pathways determining the plasma membrane sphingolipid composition are thus the key point for functional changes of the cell properties. Unnatural changes of the neuronal properties are observed in sphingolipidoses, lysosomal storage diseases occurring when a lysosomal sphingolipid hydrolase is not working, leading to the accumulation of the substrate and to its distribution to all the cell membranes interacting with lysosomes. Moreover, secondary accumulation of sphingolipids is a common trait of other lysosomal storage diseases.<\/jats:p><\/jats:sec><jats:sec><jats:label\/><jats:p>\n<jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/jnc14540-fig-0003-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text>\n<\/jats:p><jats:p><jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"https:\/\/onlinelibrary.wiley.com\/page\/journal\/14714159\/homepage\/virtual_issues.htm\">This article is part of the Special Issue \u201cLysosomal Storage Disorders\u201d<\/jats:ext-link>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jnc.14540","type":"journal-article","created":{"date-parts":[[2018,6,30]],"date-time":"2018-06-30T13:59:30Z","timestamp":1530367170000},"page":"600-611","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":40,"title":["Sphingolipids and neuronal degeneration in lysosomal storage disorders"],"prefix":"10.1111","volume":"148","author":[{"given":"Sara","family":"Grassi","sequence":"first","affiliation":[{"name":"Department of Medical Biotechnology and Translational Medicine University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7431-9207","authenticated-orcid":false,"given":"Elena","family":"Chiricozzi","sequence":"additional","affiliation":[{"name":"Department of Medical Biotechnology and Translational Medicine University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Mauri","sequence":"additional","affiliation":[{"name":"Department of Medical Biotechnology and Translational Medicine University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8180-5908","authenticated-orcid":false,"given":"Sandro","family":"Sonnino","sequence":"additional","affiliation":[{"name":"Department of Medical Biotechnology and Translational Medicine University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0252-2593","authenticated-orcid":false,"given":"Alessandro","family":"Prinetti","sequence":"additional","affiliation":[{"name":"Department of Medical Biotechnology and Translational Medicine University of Milan Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,8,16]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01049450"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.tcb.2014.07.008"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/s41419-017-0025-4"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2010.07019.x"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11064-012-0725-1"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s12035-013-8592-6"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-1-4939-1154-7_10"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbamcr.2008.12.001"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.1991.tb08281.x"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.3389\/fcell.2016.00106"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(92)90189-J"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semcdb.2015.10.031"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1432-1327.1998.2570293.x"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)37198-4"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M407749200"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10719-006-7921-7"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.7554\/eLife.00926"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature06097"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1038\/nature07596"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.201511093"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0962-8924(03)00005-9"},{"key":"e_1_2_6_23_1","first-page":"155","article-title":"Interleaflet coupling, pinning, and leaflet asymmetry\u2010major players in plasma membrane nanodomain formation","volume":"4","author":"Fujimoto T.","year":"2016","journal-title":"Front. 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