{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T19:03:47Z","timestamp":1790708627350,"version":"4.1.0"},"reference-count":34,"publisher":"Elsevier BV","issue":"4","license":[{"start":{"date-parts":[[2007,12,1]],"date-time":"2007-12-01T00:00:00Z","timestamp":1196467200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Molecular Genetics and Metabolism"],"published-print":{"date-parts":[[2007,12]]},"DOI":"10.1016\/j.ymgme.2007.08.001","type":"journal-article","created":{"date-parts":[[2007,9,19]],"date-time":"2007-09-19T15:37:36Z","timestamp":1190216256000},"page":"336-345","source":"Crossref","is-referenced-by-count":60,"title":["Secondary sphingolipid accumulation in a macrophage model of Gaucher disease"],"prefix":"10.1016","volume":"92","author":[{"given":"Leanne K.","family":"Hein","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter J.","family":"Meikle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John J.","family":"Hopwood","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Fuller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ymgme.2007.08.001_bib1","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/0006-291X(65)90743-6","article-title":"Metabolism of glucocerebrosides. II. Evidence of an enzymatic deficiency in Gaucher\u2019s disease","volume":"18","author":"Brady","year":"1965","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.ymgme.2007.08.001_bib2","doi-asserted-by":"crossref","first-page":"17C","DOI":"10.1042\/bj0970017C","article-title":"Short communications: a deficiency of glucocerebrosidase in Gaucher\u2019s disease","volume":"97","author":"Patrick","year":"1965","journal-title":"Biochem. J."},{"key":"10.1016\/j.ymgme.2007.08.001_bib3","series-title":"The Metabolic and Molecular Basis of Inherited Disease","article-title":"Gaucher disease","author":"Beutler","year":"2001"},{"key":"10.1016\/j.ymgme.2007.08.001_bib4","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1182\/blood.V33.2.379.379","article-title":"Gaucher cells in chronic myelocytic leukemia: an acquired abnormality","volume":"33","author":"Kattlove","year":"1969","journal-title":"Blood"},{"key":"10.1016\/j.ymgme.2007.08.001_bib5","doi-asserted-by":"crossref","first-page":"2529","DOI":"10.1001\/jama.237.23.2529","article-title":"Gaucher\u2019s disease in an asymptomatic 72-year-old","volume":"237","author":"Beutler","year":"1977","journal-title":"JAMA"},{"issue":"Suppl. 2","key":"10.1016\/j.ymgme.2007.08.001_bib6","first-page":"106","article-title":"Gaucher disease: understanding the molecular pathogenesis of sphingolipidoses","volume":"24","author":"Cox","year":"2001","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ymgme.2007.08.001_bib7","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/0003-2697(79)90195-7","article-title":"The distribution of glucocerebroside in the liver of patients with Gaucher\u2019s disease","volume":"95","author":"Gal","year":"1979","journal-title":"Anal. Biochem."},{"key":"10.1016\/j.ymgme.2007.08.001_bib8","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1016\/j.semcdb.2004.03.003","article-title":"The pathogenesis of glycosphingolipid storage disorders","volume":"15","author":"Ginzburg","year":"2004","journal-title":"Semin. Cell Dev. Biol."},{"key":"10.1016\/j.ymgme.2007.08.001_bib9","doi-asserted-by":"crossref","first-page":"1837","DOI":"10.1194\/jlr.M200232-JLR200","article-title":"Glucosylceramide modulates membrane traffic along the endocytic pathway","volume":"43","author":"Sillence","year":"2002","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.ymgme.2007.08.001_bib10","doi-asserted-by":"crossref","first-page":"386","DOI":"10.1038\/14084","article-title":"Cholesterol modulates membrane traffic along the endocytic pathway in sphingolipid-storage diseases","volume":"1","author":"Puri","year":"1999","journal-title":"Nat. Cell Biol."},{"key":"10.1016\/j.ymgme.2007.08.001_bib11","doi-asserted-by":"crossref","first-page":"369","DOI":"10.1083\/jcb.200106104","article-title":"Glycosphingolipids are required for sorting melanosomal proteins in the Golgi complex","volume":"155","author":"Sprong","year":"2001","journal-title":"J. Cell Biol."},{"key":"10.1016\/j.ymgme.2007.08.001_bib12","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1016\/j.bcmd.2004.03.001","article-title":"Phosphatidylcholine metabolism is altered in a monocyte-derived macrophage model of Gaucher disease but not in lymphocytes","volume":"33","author":"Trajkovic-Bodennec","year":"2004","journal-title":"Blood Cell. Mol. Dis."},{"key":"10.1016\/j.ymgme.2007.08.001_bib13","doi-asserted-by":"crossref","first-page":"23594","DOI":"10.1074\/jbc.M300212200","article-title":"Glucosylceramide and glucosylsphingosine modulate calcium mobilisation from brain microsomes via different mechanisms","volume":"278","author":"Lloyd-Evans","year":"2003","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2007.08.001_bib14","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1111\/j.1365-2141.2004.05351.x","article-title":"Gaucher disease: pathological mechanisms and modern management","volume":"129","author":"Jmoudiak","year":"2005","journal-title":"Br. J. Haematol."},{"key":"10.1016\/j.ymgme.2007.08.001_bib15","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/S0021-9258(19)52451-6","article-title":"Protein measurement with Folin Phenol reagent","volume":"193","author":"Lowry","year":"1951","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2007.08.001_bib16","doi-asserted-by":"crossref","first-page":"247","DOI":"10.1016\/0009-8981(76)90426-5","article-title":"Human leukocyte acid hydrolases: characterisation of eleven lysosomal enzymes and study of reaction conditions for their automated analysis","volume":"70","author":"Kolodny","year":"1976","journal-title":"Clin. Chim. Acta"},{"key":"10.1016\/j.ymgme.2007.08.001_bib17","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1042\/bj0780151","article-title":"Studies on glucosaminidase: IV. The fluorometric assay of N-acetyl-\u03b2-glucosaminidase","volume":"78","author":"Leaback","year":"1961","journal-title":"Biochem. J."},{"key":"10.1016\/j.ymgme.2007.08.001_bib18","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1007\/BF01086983","article-title":"Rat liver 5\u2032-nucleotidase","volume":"1","author":"El-Aaser","year":"1969","journal-title":"Histochem. J."},{"key":"10.1016\/j.ymgme.2007.08.001_bib19","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1016\/S0076-6879(55)02247-7","article-title":"Glucose-6-phosphatase from liver","volume":"2","author":"Swanson","year":"1955","journal-title":"Methods Enzymol."},{"key":"10.1016\/j.ymgme.2007.08.001_bib20","doi-asserted-by":"crossref","first-page":"497","DOI":"10.1016\/S0021-9258(18)64849-5","article-title":"A simple method for the isolation and purification of total lipids from animal tissues","volume":"226","author":"Folch","year":"1957","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.ymgme.2007.08.001_bib21","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1139\/o59-099","article-title":"A rapid method of total lipid extraction and purification","volume":"37","author":"Bligh","year":"1959","journal-title":"Can. J. Biochem. Physiol."},{"key":"10.1016\/j.ymgme.2007.08.001_bib22","doi-asserted-by":"crossref","first-page":"5510","DOI":"10.1016\/j.febslet.2006.08.041","article-title":"Genetic diseases of sphingolipid metabolism: pathological mechanisms and therapeutic options","volume":"580","author":"Kacher","year":"2006","journal-title":"FEBS Lett."},{"key":"10.1016\/j.ymgme.2007.08.001_bib23","doi-asserted-by":"crossref","first-page":"1885","DOI":"10.1016\/j.bbamem.2006.08.004","article-title":"Intracellular trafficking of sphingolipids: relationship to biosynthesis","volume":"1758","author":"Futerman","year":"2006","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgme.2007.08.001_bib24","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1194\/jlr.D200026-JLR200","article-title":"Aminopropyl solid phase extraction and 2D TLC of neutral glycosphingolipids and neutral lysoglycosphingolipids","volume":"44","author":"Bodennec","year":"2003","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.ymgme.2007.08.001_bib25","doi-asserted-by":"crossref","first-page":"643","DOI":"10.1146\/annurev.physiol.60.1.643","article-title":"Regulation of ceramide production and apoptosis","volume":"60","author":"Kolesnick","year":"1998","journal-title":"Ann. Rev. Physiol."},{"key":"10.1016\/j.ymgme.2007.08.001_bib26","doi-asserted-by":"crossref","first-page":"1814","DOI":"10.1096\/fj.02-0149fje","article-title":"Phosphatidylcholine synthesis is elevated in neuronal models of Gaucher disease due to direct activation of CTP:phosphocholine cytidylyltransferase by glucosylceramide","volume":"16","author":"Bodennec","year":"2002","journal-title":"FASEB J."},{"key":"10.1016\/j.ymgme.2007.08.001_bib27","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1034\/j.1600-0854.2003.00072.x","article-title":"Recycling compartments and the internal vesicles of multivesicular bodies harbour most of the cholesterol found in the endocytic pathway","volume":"4","author":"Mobius","year":"2003","journal-title":"Traffic"},{"key":"10.1016\/j.ymgme.2007.08.001_bib28","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1042\/BJ20050325","article-title":"The N370S (Asn370\u2192Ser) mutation affects the capacity of glucosylceramidase to interact with anionic phospholipid-containing membranes and saposin C","volume":"390","author":"Salvioli","year":"2005","journal-title":"Biochem. J."},{"key":"10.1016\/j.ymgme.2007.08.001_bib29","doi-asserted-by":"crossref","first-page":"1182","DOI":"10.1172\/JCI110363","article-title":"Selective effects of glucocerebroside (Gaucher\u2019s storage material) on macrophage cultures","volume":"68","author":"Gery","year":"1981","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.ymgme.2007.08.001_bib30","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/0005-2760(82)90272-7","article-title":"The occurrence of psychosine and other glycolipids in spleen and liver from the three major types of Gaucher\u2019s disease","volume":"712","author":"Nilsson","year":"1982","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgme.2007.08.001_bib31","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1016\/S0022-2275(20)38985-9","article-title":"Lipids of the spleen in Gaucher\u2019s disease","volume":"6","author":"Suomi","year":"1965","journal-title":"J. Lipid Res."},{"key":"10.1016\/j.ymgme.2007.08.001_bib32","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1016\/0006-291X(64)90503-0","article-title":"Isolation and characterisation of the main splenic glycolipids in Gaucher\u2019s disease: evidence for the site of metabolic block","volume":"15","author":"Philippart","year":"1964","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"10.1016\/j.ymgme.2007.08.001_bib33","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1016\/0005-2760(70)90079-2","article-title":"Sphingolipid hydrolases in brain tissue of patients with generalised gangliosidosis","volume":"210","author":"Brady","year":"1970","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.ymgme.2007.08.001_bib34","doi-asserted-by":"crossref","first-page":"459","DOI":"10.1016\/S0962-8924(00)01847-X","article-title":"Jamming the endosomal system: lipid rafts and lysosomal storage diseases","volume":"10","author":"Simons","year":"2000","journal-title":"Trends Cell Biol."}],"container-title":["Molecular Genetics and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719207002521?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719207002521?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2021,8,24]],"date-time":"2021-08-24T00:00:23Z","timestamp":1629763223000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1096719207002521"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,12]]},"references-count":34,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2007,12]]}},"alternative-id":["S1096719207002521"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgme.2007.08.001","relation":{},"ISSN":["1096-7192"],"issn-type":[{"value":"1096-7192","type":"print"}],"subject":[],"published":{"date-parts":[[2007,12]]}}}