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In our previous report we isolated membranes of the LE\/LY compartment from NPC L1 skin fibroblasts with a mutation in the NPC1 gene (exon 8, R348X) and showed that annexin A6 (AnxA6) may contribute to the impaired dynamics of these membranes in a cholesterol-dependent manner and therefore to the overnormative storage of cholesterol. In this report we show that the LE\/LY fraction isolated from NPC L1 cells is characterized by a 4-fold enrichment in cholesterol, 2.5-fold in sphingomyelin and 2-fold in saturated fatty acids. As a result, the fluidity of LE\/LY membranes isolated from NPC L1 cells is greatly reduced in comparison to control ones. We conclude that modified lipid composition and properties of this compartment may affect distribution and function of proteins implicated in cellular membrane dynamics. As a consequence, the backward vesicular transport of cholesterol from the LE\/LY compartment to the Golgi apparatus, endoplasmic reticulum and finally to plasma membrane is impaired.<\/jats:p>\n                  <jats:p\/>","DOI":"10.1039\/c2mb05447g","type":"journal-article","created":{"date-parts":[[2012,1,27]],"date-time":"2012-01-27T16:39:46Z","timestamp":1327682386000},"page":"1197-1205","source":"Crossref","is-referenced-by-count":21,"title":["Impaired dynamics of the late endosome\/lysosome compartment in human Niemann\u2013Pick type C skin fibroblasts carrying mutation in\n                    <i>NPC1<\/i>\n                    gene"],"prefix":"10.1093","volume":"8","author":[{"given":"Malgorzata E","family":"Sztolsztener","sequence":"first","affiliation":[{"name":"Department of Biochemistry, Nencki Institute of Experimental Biology, Polish Academy of Sciences a , 3 Pasteur Street, PL-02093 Warsaw Poland \u00a0 j.bandorowicz-pikula@nencki.gov.pl"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Agnieszka","family":"Dobrzyn","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Nencki Institute of Experimental Biology, Polish Academy of Sciences a , 3 Pasteur Street, PL-02093 Warsaw Poland \u00a0 j.bandorowicz-pikula@nencki.gov.pl"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Slawomir","family":"Pikula","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Nencki Institute of Experimental Biology, Polish Academy of Sciences a , 3 Pasteur Street, PL-02093 Warsaw Poland \u00a0 j.bandorowicz-pikula@nencki.gov.pl"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Tylki-Szymanska","sequence":"additional","affiliation":[{"name":"Clinic of Metabolic Diseases, Endocrinology and Diabetology, The Children's Memorial Health Institute b , Warsaw, Poland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joanna","family":"Bandorowicz-Pikula","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Nencki Institute of Experimental Biology, Polish Academy of Sciences a , 3 Pasteur Street, PL-02093 Warsaw Poland \u00a0 j.bandorowicz-pikula@nencki.gov.pl"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2012,1,27]]},"reference":[{"key":"2026012913035123800_cit1","doi-asserted-by":"crossref","first-page":"609","DOI":"10.1074\/jbc.M108861200","volume":"277","author":"Hao","year":"2002","journal-title":"J. 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