{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,3,27]],"date-time":"2024-03-27T23:44:27Z","timestamp":1711583067935},"reference-count":26,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[1987,10,28]],"date-time":"1987-10-28T00:00:00Z","timestamp":562377600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1988,6]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Epstein\u2010Barr virus\u2010transformed lymphoid cell lines from normal individuals and from patients with Niemann\u2010Pick disease types A, B or C were subjected to various culture conditions in order to study the source of the characteristic lysosomal storage of sphingomyelin observed in the tissues of Niemann\u2010Pick patients. The culture medium was supplemented with a serum substitute devoid of lipoproteins or with one of the following lipid sources: fetal calf serum, human low\u2010density lipoprotein (LDL), or human high\u2010density lipoprotein (HDL). Storage of sphingomyelin was demonstrated under all tested culture conditions in cells deficient in acid sphingomyelinase (Niemann\u2010Pick disease types A and B). In contrast, the sphingomyelin concentration in the lymphoid cell line from a Niemann\u2010Pick type C patient (not deficient in sphingomyelinase) was normal. After more than 30 days in a medium devoid of sphingomyelin, the Niemann\u2010Pick types A and B lymphoid cell lines showed accumulation of sphingomyelin about twice control. The concentration was higher when cells were grown in a medium supplemented with lipids, particularly human LDL or HDL. These results are consistent with the hypothesis that both exogenous and endogenous sphingomyelins participate in the lysosomal storage observed in lymphoid cell lines from patients with Niemann\u2010Pick disease types A and B.<\/jats:p>","DOI":"10.1007\/bf01799864","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T16:40:50Z","timestamp":1119372050000},"page":"151-157","source":"Crossref","is-referenced-by-count":4,"title":["Evidence for both endogenous and exogenous sources of the sphingomyelin storage in lymphoid cell lines from patients with Niemann\u2010Pick disease types A and B"],"prefix":"10.1002","volume":"11","author":[{"given":"T.","family":"Levade","sequence":"first","affiliation":[{"name":"INSERM Unit\u00e9 101 et Laboratoire de Biochimie M\u00e9dicale, Facult\u00e9 de M\u00e9decine Purpan  37 All\u00e9es Jules Guesde Toulouse Cedex F\u201031073 France"}]},{"given":"R.","family":"Salvayre","sequence":"additional","affiliation":[{"name":"INSERM Unit\u00e9 101 et Laboratoire de Biochimie M\u00e9dicale, Facult\u00e9 de M\u00e9decine Purpan  37 All\u00e9es Jules Guesde Toulouse Cedex F\u201031073 France"}]},{"given":"A.","family":"Maret","sequence":"additional","affiliation":[{"name":"INSERM Unit\u00e9 101 et Laboratoire de Biochimie M\u00e9dicale, Facult\u00e9 de M\u00e9decine Purpan  37 All\u00e9es Jules Guesde Toulouse Cedex F\u201031073 France"}]},{"given":"L.","family":"Douste\u2010Blazy","sequence":"additional","affiliation":[{"name":"INSERM Unit\u00e9 101 et Laboratoire de Biochimie M\u00e9dicale, Facult\u00e9 de M\u00e9decine Purpan  37 All\u00e9es Jules Guesde Toulouse Cedex F\u201031073 France"}]}],"member":"311","published-online":{"date-parts":[[1987,10,28]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0076-6879(66)08014-5"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0006-291X(82)80004-1"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1768-322X.1984.tb00277.x"},{"key":"e_1_2_1_5_1","first-page":"831","volume-title":"The Metabolic Basis of Inherited Disease","author":"Brady R. 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