{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T23:32:41Z","timestamp":1791329561237,"version":"4.3.1"},"reference-count":30,"publisher":"Elsevier BV","issue":"3","license":[{"start":{"date-parts":[[2011,9,1]],"date-time":"2011-09-01T00:00:00Z","timestamp":1314835200000},"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":["Biochemical and Biophysical Research Communications"],"published-print":{"date-parts":[[2011,9]]},"DOI":"10.1016\/j.bbrc.2011.08.096","type":"journal-article","created":{"date-parts":[[2011,9,3]],"date-time":"2011-09-03T16:30:48Z","timestamp":1315067448000},"page":"400-406","source":"Crossref","is-referenced-by-count":46,"title":["Npc1 deficiency in the C57BL\/6J genetic background enhances Niemann\u2013Pick disease type C spleen pathology"],"prefix":"10.1016","volume":"413","author":[{"given":"Julio","family":"Parra","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andr\u00e9s D.","family":"Klein","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Juan","family":"Castro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda Gabriela","family":"Morales","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mat\u00edas","family":"Mosqueira","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilse","family":"Valencia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Victor","family":"Cort\u00e9s","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Attilio","family":"Rigotti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Silvana","family":"Zanlungo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.bbrc.2011.08.096_b0005","series-title":"The Metabolic and Molecular Basis of Inherited Disease","first-page":"1","article-title":"Niemann\u2013Pick disease type C. A lipid trafficking disorder","author":"Patterson","year":"2001"},{"key":"10.1016\/j.bbrc.2011.08.096_b0010","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1097\/00041433-199804000-00009","article-title":"Niemann\u2013Pick disease type C","volume":"9","author":"Liscum","year":"1998","journal-title":"Curr. Opin. Lipidol."},{"key":"10.1016\/j.bbrc.2011.08.096_b0015","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1126\/science.277.5323.228","article-title":"Niemann\u2013Pick C1 disease gene: homology to mediators of cholesterol homeostasis","volume":"277","author":"Cartsea","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.bbrc.2011.08.096_b0020","doi-asserted-by":"crossref","first-page":"2298","DOI":"10.1126\/science.290.5500.2298","article-title":"Identification of HE1 as the second gene of Niemann\u2013Pick C disease","volume":"290","author":"Naureckiene","year":"2000","journal-title":"Science"},{"key":"10.1016\/j.bbrc.2011.08.096_b0025","doi-asserted-by":"crossref","first-page":"1064","DOI":"10.1074\/jbc.M707944200","article-title":"Purified NPC1 protein. II. Localization of sterol binding to a 240-amino acid soluble luminal loop","volume":"283","author":"Infante","year":"2008","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbrc.2011.08.096_b0030","doi-asserted-by":"crossref","first-page":"1213","DOI":"10.1016\/j.cell.2009.03.049","article-title":"Structure of N-Terminal domain of NPC1 reveals distinct subdomains for binding and transfer of cholesterol","volume":"137","author":"Kwon","year":"2009","journal-title":"Cell"},{"key":"10.1016\/j.bbrc.2011.08.096_b0035","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1016\/j.bbalip.2004.08.008","article-title":"Intracellular trafficking of Niemann\u2013Pick C proteins 1 and 2: obligate components of subcellular lipid transport","volume":"1685","author":"Liscum","year":"2004","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbrc.2011.08.096_b0040","doi-asserted-by":"crossref","first-page":"20917","DOI":"10.1074\/jbc.R400040200","article-title":"Niemann\u2013Pick type C disease and intracellular cholesterol trafficking","volume":"280","author":"Chang","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbrc.2011.08.096_b0045","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1034\/j.1399-0004.2003.00147.x","article-title":"Niemann\u2013Pick disease type C","volume":"64","author":"Vanier","year":"2003","journal-title":"Clin. Genet."},{"key":"10.1016\/j.bbrc.2011.08.096_b0050","doi-asserted-by":"crossref","first-page":"1204","DOI":"10.1002\/ajmg.a.31735","article-title":"The National Niemann\u2013Pick C1 disease database: report of clinical features and health problems","volume":"143","author":"Garver","year":"2007","journal-title":"Am. J. Med. Genet. A"},{"key":"10.1016\/j.bbrc.2011.08.096_b0055","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1016\/0005-2760(80)90116-2","article-title":"A lysosomal storage disorder in mice characterized by a dual deficiency of sphingomyelinase and glucocerebrosidase","volume":"619","author":"Pentchev","year":"1980","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbrc.2011.08.096_b0060","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1093\/oxfordjournals.jhered.a109635","article-title":"Sphingomyelinosis, a new mutation in the mouse: a model of Niemann\u2013Pick disease in humans","volume":"73","author":"Miyawaki","year":"1982","journal-title":"J. Hered."},{"key":"10.1016\/j.bbrc.2011.08.096_b0065","first-page":"140","article-title":"Lysosome lipid storage disorder in NCTR-BALB\/c mice. I. Description of the disease and genetics","volume":"108","author":"Morris","year":"1982","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.bbrc.2011.08.096_b0070","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1007\/s004390050370","article-title":"A C57BL\/KsJ mouse model of Niemann\u2013Pick disease (spm) belongs to the same complementation group as the major childhood type of Niemann\u2013Pick disease type C","volume":"99","author":"Akaboshi","year":"1997","journal-title":"Hum. Genet."},{"key":"10.1016\/j.bbrc.2011.08.096_b0075","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1126\/science.277.5323.232","article-title":"Murine model of Niemann\u2013Pick C disease: mutation in a cholesterol homeostasis gene","volume":"277","author":"Loftus","year":"1997","journal-title":"Science"},{"key":"10.1016\/j.bbrc.2011.08.096_b0080","doi-asserted-by":"crossref","first-page":"5784","DOI":"10.1016\/S0021-9258(18)91082-3","article-title":"A genetic storage disorder in BALB\/c mice with a metabolic block in sterification of exogenous cholesterol","volume":"259","author":"Pentchev","year":"1984","journal-title":"J. Biol. Chem."},{"key":"10.1016\/j.bbrc.2011.08.096_b0085","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.bbalip.2004.08.011","article-title":"Consequences of NPC1 and NPC2 loss of function in mammalian neurons","volume":"1685","author":"Walkley","year":"2004","journal-title":"Biochim. Biophys. Acta"},{"key":"10.1016\/j.bbrc.2011.08.096_b0090","doi-asserted-by":"crossref","first-page":"3617","DOI":"10.1096\/fj.07-102715","article-title":"Imatinib therapy blocks cerebellar apoptosis and improves neurological symptoms in a mouse model of Niemann\u2013Pick type C disease","volume":"10","author":"Alvarez","year":"2008","journal-title":"FASEB J."},{"key":"10.1016\/j.bbrc.2011.08.096_b0095","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1053\/jhep.2002.35617","article-title":"Relevance of Niemann\u2013Pick type C1 protein expression in controlling plasma cholesterol and biliary lipid secretion in mice","volume":"36","author":"Amigo","year":"2002","journal-title":"Hepatology"},{"key":"10.1016\/j.bbrc.2011.08.096_b0100","doi-asserted-by":"crossref","first-page":"1708","DOI":"10.1053\/gast.2000.20198","article-title":"Overexpression of sterol carrier protein-2 leads to altered lipid metabolism and enhanced enterohepatic sterol circulation in mice","volume":"119","author":"Zanlungo","year":"2000","journal-title":"Gastroenterology"},{"key":"10.1016\/j.bbrc.2011.08.096_b0105","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.bbrc.2011.08.096_b0110","doi-asserted-by":"crossref","first-page":"1818","DOI":"10.1172\/JCI113797","article-title":"Regulation of biliary cholesterol secretion. Functional relationship between the canalicular and sinusoidal cholesterol secretory pathways in the rat","volume":"82","author":"Nervi","year":"1988","journal-title":"J. Clin. Invest."},{"key":"10.1016\/j.bbrc.2011.08.096_b0115","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1093\/jnen\/64.4.323","article-title":"Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann\u2013Pick type C disease","volume":"64","author":"Li","year":"2005","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1016\/j.bbrc.2011.08.096_b0120","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1016\/S0065-1281(11)80139-X","article-title":"Niemann Pick c or storage by excessive blood cell destruction: a case presenting a diagnosis problem","volume":"92","author":"Lageron","year":"1992","journal-title":"Acta Histochem."},{"key":"10.1016\/j.bbrc.2011.08.096_b0125","doi-asserted-by":"crossref","first-page":"446","DOI":"10.1002\/ajmg.a.32642","article-title":"The clinical spectrum of fetal Niemann\u2013Pick type C","volume":"149A","author":"Spiegel","year":"2009","journal-title":"Am. J. Med. Genet. A"},{"key":"10.1016\/j.bbrc.2011.08.096_b0130","doi-asserted-by":"crossref","first-page":"3107","DOI":"10.1093\/hmg\/11.24.3107","article-title":"Rescue of neurodegeneration in Niemann\u2013Pick C mice by a prion-promoter-driven Npc1 cDNA transgene","volume":"11","author":"Loftus","year":"2002","journal-title":"Hum. Mol. Genet."},{"key":"10.1016\/j.bbrc.2011.08.096_b0135","first-page":"373","article-title":"A fatal respiratory form of type C Niemann\u2013Pick disease","volume":"47","author":"Pin","year":"1990","journal-title":"Arch. Fr. Pediatr."},{"key":"10.1016\/j.bbrc.2011.08.096_b0140","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1038\/sj.bmt.1701826","article-title":"Niemann\u2013Pick disease type C (a cellular cholesterol lipidosis) treated by bone marrow transplantation","volume":"24","author":"Hsu","year":"1999","journal-title":"Bone Marrow Transplant."},{"key":"10.1016\/j.bbrc.2011.08.096_b0145","doi-asserted-by":"crossref","first-page":"23","DOI":"10.1385\/MO:23:1:23","article-title":"The diagnostic plot: a concept for identifying different states of iron deficiency and monitoring the response to epoetin therapy","volume":"23","author":"Thomas","year":"2006","journal-title":"Rev. Med. Oncol."},{"key":"10.1016\/j.bbrc.2011.08.096_b0150","doi-asserted-by":"crossref","first-page":"4367","DOI":"10.1523\/JNEUROSCI.5981-10.2011","volume":"12","author":"Lopez","year":"2011","journal-title":"J. Neurosci."}],"container-title":["Biochemical and Biophysical Research Communications"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006291X11015051?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0006291X11015051?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2021,12,3]],"date-time":"2021-12-03T04:27:13Z","timestamp":1638505633000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0006291X11015051"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,9]]},"references-count":30,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2011,9]]}},"alternative-id":["S0006291X11015051"],"URL":"https:\/\/doi.org\/10.1016\/j.bbrc.2011.08.096","relation":{},"ISSN":["0006-291X"],"issn-type":[{"value":"0006-291X","type":"print"}],"subject":[],"published":{"date-parts":[[2011,9]]}}}