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The storage was represented by a crescent\u2010shaped line of storage lysosomes of varying thicknesses restricted to the perinuclear subplasmalemmal area. The ultrastructure of the storage lysosomes was dominated by concentric lipid membranes modified by simultaneous deposition of autofluorescent ceroid. Storage was widely expressed in adipose tissue. The number of storage lysosomes was increased, and the lysosomes were more clustered in adipocytes with less voluminous lipid content. The findings should attract interest to studies of adipose tissue biology in Fabry disease, a topic that has not been studied so far. In terms of cell biology, the observations represent indirect evidence of significant lysosomal turnover of \u03b1\u2010galactose lipid conjugates in adipocytes demasked by GLA deficiency. The results extend the thus far limited information on the adipocyte lysosomal system and its participation in lysosomal storage disorders.<\/jats:p>","DOI":"10.1007\/s10545-010-9160-0","type":"journal-article","created":{"date-parts":[[2010,7,13]],"date-time":"2010-07-13T09:32:24Z","timestamp":1279013544000},"page":"297-300","source":"Crossref","is-referenced-by-count":1,"title":["Adipocytes participate in storage in \u03b1\u2010galactosidase deficiency (Fabry disease)"],"prefix":"10.1002","volume":"33","author":[{"given":"Helena","family":"H\u016flkov\u00e1","sequence":"first","affiliation":[{"name":"Institute of Inherited Metabolic Disorders Charles University, 1st Faculty of Medicine and Teaching Hospital Prague Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Milan","family":"Elleder","sequence":"additional","affiliation":[{"name":"Institute of Inherited Metabolic Disorders Charles University, 1st Faculty of Medicine and Teaching Hospital Prague Czech Republic"},{"name":"Institute of Inherited Metabolic Disorders Division B, Bldg.D, Ke Karlovu 2 Prague 128 08 Czech Republic"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2010,7,14]]},"reference":[{"key":"e_1_2_8_2_1","first-page":"1440","article-title":"Changes in lysosomal enzyme activity in the process of L\u2010cell adipocyte differentiation","volume":"24","author":"Borisov AB","year":"1982","journal-title":"Tsitologiia"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1006\/rwgn.2001.0443"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-2275(20)31157-3"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02890291"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-90-481-9033-1_3"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1210\/en.2008-1685"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-009-1178-9"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00428-008-0586-9"},{"key":"e_1_2_8_10_1","first-page":"39","article-title":"Receptor mediated insulin degradation decreased by chloroquine in isolated rat adipocytes","volume":"88","author":"Kobayashi M","year":"1980","journal-title":"J Biochem"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M702223200"},{"key":"e_1_2_8_12_1","first-page":"78","article-title":"Fabry's disease without cutaneous angiokeratoma : diagnosis by electron microscope study of skin biopsy (author's transl)","volume":"57","author":"Le Charpentier Y","year":"1981","journal-title":"Sem H\u00f4p"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0005-2760(96)00175-0"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1194\/jlr.M600065-JLR200"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02091541"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1083\/jcb.87.1.180"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M006739200"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.1320570213"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neurobiolaging.2005.12.006"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.79.23.7302"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M508020200"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-010-9160-0","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T06:19:56Z","timestamp":1694758796000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-010-9160-0"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,7,14]]},"references-count":20,"journal-issue":{"issue":"S3","published-print":{"date-parts":[[2010,12]]}},"alternative-id":["10.1007\/s10545-010-9160-0"],"URL":"https:\/\/doi.org\/10.1007\/s10545-010-9160-0","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,7,14]]}}}