{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T02:00:39Z","timestamp":1791165639728,"version":"4.1.0"},"reference-count":101,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":2001,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"DOI":"10.1111\/j.1600-0854.2010.01032.x","type":"journal-article","created":{"date-parts":[[2010,1,6]],"date-time":"2010-01-06T13:05:34Z","timestamp":1262783134000},"page":"419-428","source":"Crossref","is-referenced-by-count":177,"title":["Lipids on Trial: The Search for the Offending Metabolite in Niemann-Pick type C Disease"],"prefix":"10.1111","volume":"11","author":[{"given":"Emyr","family":"Lloyd-Evans","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Frances M.","family":"Platt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,3,10]]},"reference":[{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB1|cit1","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1023\/A:1022575511354","article-title":"Lipid changes in Niemann-Pick disease type C brain: personal experience and review of the literature","volume":"24","author":"Vanier","year":"1999","journal-title":"Neurochem Res"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB2|cit2","first-page":"81","article-title":"Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease","volume":"1","author":"Ko","year":"2005","journal-title":"PLoS Genet"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB3|cit3","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1097\/01.nrl.0000094627.78754.5b","article-title":"A riddle wrapped in a mystery: understanding Niemann-Pick disease, type C","volume":"9","author":"Patterson","year":"2003","journal-title":"Neurologist"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB4|cit4","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1093\/brain\/118.1.119","article-title":"Neurofibrillary tangles in Niemann-Pick disease type C","volume":"118","author":"Love","year":"1995","journal-title":"Brain"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB5|cit5","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1016\/S0002-9440(10)63163-X","article-title":"Niemann-Pick Type C disease and Alzheimer's disease: the APP-endosome connection fattens up","volume":"164","author":"Nixon","year":"2004","journal-title":"Am J Pathol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB6|cit6","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1016\/j.tibs.2005.07.007","article-title":"Guilty until proven innocent: the case of NPC1 and cholesterol","volume":"30","author":"Ioannou","year":"2005","journal-title":"Trends Biochem Sci"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB7|cit7","volume-title":"The Metabolic & Molecular Bases of Inherited Disease","author":"Scriver","year":"2001"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB8|cit8","doi-asserted-by":"crossref","first-page":"5886","DOI":"10.1073\/pnas.0308456101","article-title":"Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport","volume":"101","author":"Sleat","year":"2004","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB9|cit9","doi-asserted-by":"crossref","first-page":"3132","DOI":"10.1091\/mbc.e04-02-0103","article-title":"Disturbed cholesterol traffic but normal proteolytic function in LAMP-1\/LAMP-2 double-deficient fibroblasts","volume":"15","author":"Eskelinen","year":"2004","journal-title":"Mol Biol Cell"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB10|cit10","doi-asserted-by":"crossref","first-page":"12081","DOI":"10.1074\/jbc.M600621200","article-title":"The lipoprotein receptor-related protein-1 (LRP) adapter protein GULP mediates trafficking of the LRP ligand prosaposin, leading to sphingolipid and free cholesterol accumulation in late endosomes and impaired efflux","volume":"281","author":"Kiss","year":"2006","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB11|cit11","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1515\/BC.2007.063","article-title":"Development of an assay for the intermembrane transfer of cholesterol by Niemann-Pick C2 protein","volume":"388","author":"Babalola","year":"2007","journal-title":"Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB12|cit12","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":"10.1111\/j.1600-0854.2010.01032.x-BIB13|cit13","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1016\/S0960-9822(01)00178-6","article-title":"The sterol-sensing domain of patched protein seems to control smoothened activity through patched vesicular trafficking","volume":"11","author":"Martin","year":"2001","journal-title":"Curr Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB14|cit14","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1097\/00041433-199804000-00010","article-title":"Related membrane domains in proteins of sterol sensing and cell signaling provide a glimpse of treasures still buried within the dynamic realm of intracellular metabolic regulation","volume":"9","author":"Osborne","year":"1998","journal-title":"Curr Opin Lipidol"},{"issue":"11","key":"10.1111\/j.1600-0854.2010.01032.x-BIB15|cit15","doi-asserted-by":"crossref","first-page":"1247","DOI":"10.1038\/nm.1876","article-title":"Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium","volume":"14","author":"Lloyd-Evans","year":"2008 Nov","journal-title":"Nat Med"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB16|cit16","doi-asserted-by":"crossref","first-page":"26167","DOI":"10.1074\/jbc.M311591200","article-title":"Accumulation of glycosphingolipids in Niemann-Pick C disease disrupts endosomal transport","volume":"279","author":"Te Vruchte","year":"2004","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB17|cit17","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1091\/mbc.12.3.601","article-title":"Dynamic movements of organelles containing Niemann-Pick C1 protein: NPC1 involvement in late endocytic events","volume":"12","author":"Ko","year":"2001","journal-title":"Mol Biol Cell"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB18|cit18","doi-asserted-by":"crossref","first-page":"3242","DOI":"10.1093\/emboj\/cdg321","article-title":"Annexin II regulates multivesicular endosome biogenesis in the degradation pathway of animal cells","volume":"22","author":"Mayran","year":"2003","journal-title":"Embo J"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB19|cit19","first-page":"77","article-title":"Membrane traffic to and from lysosomes","volume":"2005","author":"Luzio","journal-title":"Biochem Soc Symp"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB20|cit20","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1006\/bbrc.1993.2228","article-title":"Niemann-Pick C disease: cystine and lipids accumulate in the murine model of this lysosomal cholesterol lipidosis","volume":"196","author":"Butler","year":"1993","journal-title":"Biochem Biophys Res Commun"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB21|cit21","doi-asserted-by":"crossref","first-page":"303","DOI":"10.1016\/0005-2760(92)90236-O","article-title":"Type C Niemann-Pick disease: a murine model of the lysosomal cholesterol lipidosis accumulates sphingosine and sphinganine in liver","volume":"1127","author":"Goldin","year":"1992","journal-title":"Biochim Biophys Acta"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB22|cit22","doi-asserted-by":"crossref","first-page":"8247","DOI":"10.1073\/pnas.82.23.8247","article-title":"A defect in cholesterol esterification in Niemann-Pick disease (type C) patients","volume":"82","author":"Pentchev","year":"1985","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB23|cit23","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1016\/0925-4439(94)90021-3","article-title":"Free sphingoid bases in tissues from patients with type C Niemann-Pick disease and other lysosomal storage disorders","volume":"1226","author":"Rodriguez-Lafrasse","year":"1994","journal-title":"Biochim Biophys Acta"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB24|cit24","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1007\/BF00690966","article-title":"Niemann-Pick disease type C. Study on the nature of the cerebral storage process.","volume":"66","author":"Elleder","year":"1985","journal-title":"Acta Neuropathol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB25|cit25","doi-asserted-by":"crossref","first-page":"4168","DOI":"10.1074\/jbc.M205406200","article-title":"Trafficking of cholesterol from cell bodies to distal axons in Niemann Pick C1-deficient neurons","volume":"278","author":"Karten","year":"2003","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB26|cit26","doi-asserted-by":"crossref","first-page":"3138","DOI":"10.1021\/bi00434a004","article-title":"Structural requirements for long-chain (sphingoid) base inhibition of protein kinase C in vitro and for the cellular effects of these compounds","volume":"28","author":"Merrill","year":"1989","journal-title":"Biochemistry"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB27|cit27","doi-asserted-by":"crossref","first-page":"36710","DOI":"10.1074\/jbc.M608743200","article-title":"NPC2, the protein deficient in Niemann-Pick C2 disease, consists of multiple glycoforms that bind a variety of sterols","volume":"281","author":"Liou","year":"2006","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB28|cit28","doi-asserted-by":"crossref","first-page":"1625","DOI":"10.1083\/jcb.108.5.1625","article-title":"The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts","volume":"108","author":"Liscum","year":"1989","journal-title":"J Cell Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB29|cit29","doi-asserted-by":"crossref","first-page":"17002","DOI":"10.1016\/S0021-9258(18)45483-X","article-title":"Low density lipoprotein (LDL)-mediated suppression of cholesterol synthesis and LDL uptake is defective in Niemann-Pick type C fibroblasts","volume":"262","author":"Liscum","year":"1987","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB30|cit30","doi-asserted-by":"crossref","first-page":"41309","DOI":"10.1074\/jbc.M008272200","article-title":"Fate of endogenously synthesized cholesterol in Niemann-Pick type C1 cells","volume":"275","author":"Cruz","year":"2000","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB31|cit31","doi-asserted-by":"crossref","first-page":"17674","DOI":"10.1074\/jbc.M313517200","article-title":"Glucosylceramidase mass and subcellular localization are modulated by cholesterol in Niemann-Pick disease type C","volume":"279","author":"Salvioli","year":"2004","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB32|cit32","doi-asserted-by":"crossref","first-page":"120","DOI":"10.1016\/j.abb.2003.08.031","article-title":"Elevation of ceramide in serum lipoproteins during acute phase response in humans and mice: role of serine-palmitoyl transferase","volume":"419","author":"Lightle","year":"2003","journal-title":"Arch Biochem Biophys"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB33|cit33","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1093\/glycob\/11.10.791","article-title":"Characterization of human apolipoprotein B100 oligosaccharides in LDL subfractions derived from normal and hyperlipidemic plasma: deficiency of alpha-N-acetylneuraminyllactosyl-ceramide in light and small dense LDL particles","volume":"11","author":"Garner","year":"2001","journal-title":"Glycobiology"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB34|cit34","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1083\/jcb.200205034","article-title":"Mycobacterium and the coat of many lipids","volume":"158","author":"Russell","year":"2002","journal-title":"J Cell Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB35|cit35","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1016\/j.cbi.2009.04.012","article-title":"Transport of lipids by ABC proteins: interactions and implications for cellular toxicity, viability and function","volume":"180","author":"Aye","year":"2009","journal-title":"Chem Biol Interact"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB36|cit36","doi-asserted-by":"crossref","first-page":"4138","DOI":"10.1128\/MCB.25.10.4138-4149.2005","article-title":"ABCA5 resides in lysosomes, and ABCA5 knockout mice develop lysosomal disease-like symptoms","volume":"25","author":"Kubo","year":"2005","journal-title":"Mol Cell Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB37|cit37","doi-asserted-by":"crossref","first-page":"849","DOI":"10.1523\/JNEUROSCI.21-03-00849.2001","article-title":"Atp-binding cassette transporter ABC2\/ ABCA2 in the rat brain: a novel mammalian lysosome-associated membrane protein and a specific marker for oligodendrocytes but not for myelin sheaths","volume":"21","author":"Zhou","year":"2001","journal-title":"J Neurosci"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB38|cit38","doi-asserted-by":"crossref","first-page":"1558","DOI":"10.1096\/fj.04-2714fje","article-title":"Protein transduction of Rab9 in Niemann-Pick C cells reduces cholesterol storage","volume":"19","author":"Narita","year":"2005","journal-title":"Faseb J"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB39|cit39","article-title":"Endosomal lipid accumulation in NPC1 leads to inhibition of PKC, hypophosphorylation of vimentin and Rab9 entrapment","author":"Walter","year":"2008","journal-title":"Biol Cell"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB40|cit40","article-title":"MLN64 mediates egress of cholesterol from endosomes to mitochondria in the absence of functional Niemann-Pick Type C1 protein","author":"Charman","year":"2009","journal-title":"J Lipid Res"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB41|cit41","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.0910916106","article-title":"Cyclodextrin overcomes deficient lysosome-to-endoplasmic reticulum transport of cholesterol in Niemann-Pick type C cells","author":"Abi-Mosleh","year":"2009","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB42|cit42","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0006951","article-title":"Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression","volume":"4","author":"Davidson","year":"2009","journal-title":"PLoS One"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB43|cit43","doi-asserted-by":"crossref","first-page":"17468","DOI":"10.1074\/jbc.M000875200","article-title":"Cholesterol movement in Niemann-Pick type C cells and in cells treated with amphiphiles","volume":"275","author":"Lange","year":"2000","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB44|cit44","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1212\/WNL.43.1_Part_1.61","article-title":"The effect of cholesterol-lowering agents on hepatic and plasma cholesterol in Niemann-Pick disease type C","volume":"43","author":"Patterson","year":"1993","journal-title":"Neurology"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB45|cit45","doi-asserted-by":"crossref","first-page":"427","DOI":"10.1023\/A:1010588112003","article-title":"Effects of dietary cholesterol restriction in a feline model of Niemann-Pick type C disease","volume":"24","author":"Somers","year":"2001","journal-title":"J Inherit Metab Dis"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB46|cit46","doi-asserted-by":"crossref","first-page":"54","DOI":"10.1023\/A:1005650930330","article-title":"Pharmacological and genetic modifications of somatic cholesterol do not substantially alter the course of CNS disease in Niemann-Pick C mice","volume":"23","author":"Erickson","year":"2000","journal-title":"J Inherit Metab Dis"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB47|cit47","doi-asserted-by":"crossref","first-page":"1361","DOI":"10.1086\/320599","article-title":"Niemann-Pick C variant detection by altered sphingolipid trafficking and correlation with mutations within a specific domain of NPC1","volume":"68","author":"Sun","year":"2001","journal-title":"Am J Hum Genet"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB48|cit48","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1194\/jlr.M200230-JLR200","article-title":"Telomerase immortalization upregulates Rab9 expression and restores LDL cholesterol egress from Niemann-Pick C1 late endosomes","volume":"44","author":"Walter","year":"2003","journal-title":"J Lipid Res"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB49|cit49","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1042\/BC20070171","article-title":"Endosomal lipid accumulation in NPC1 leads to inhibition of PKC, hypophosphorylation of vimentin and Rab9 entrapment","volume":"101","author":"Walter","year":"2009","journal-title":"Biol Cell"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB50|cit50","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1006\/bbrc.1994.1064","article-title":"The attenuated elevation of cytoplasmic calcium concentration following the uptake of low density lipoprotein in type C Niemann-Pick fibroblasts","volume":"198","author":"Yamamoto","year":"1994","journal-title":"Biochem Biophys Res Commun"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB51|cit51","doi-asserted-by":"crossref","first-page":"1708","DOI":"10.1194\/jlr.M200179-JLR200","article-title":"Identification of a pharmaceutical compound that partially corrects the Niemann-Pick C phenotype in cultured cells","volume":"43","author":"Liscum","year":"2002","journal-title":"J Lipid Res"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB52|cit52","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1083\/jcb.200110138","article-title":"Overexpression of the myelin proteolipid protein leads to accumulation of cholesterol and proteolipid protein in endosomes\/lysosomes: implications for Pelizaeus-Merzbacher disease","volume":"157","author":"Simons","year":"2002","journal-title":"J Cell Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB53|cit53","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":"10.1111\/j.1600-0854.2010.01032.x-BIB54|cit54","doi-asserted-by":"crossref","first-page":"538","DOI":"10.1165\/rcmb.2004-0117OC","article-title":"Mechanistic similarities between cultured cell models of cystic fibrosis and niemann-pick type C","volume":"31","author":"White","year":"2004","journal-title":"Am J Respir Cell Mol Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB55|cit55","doi-asserted-by":"crossref","first-page":"3578","DOI":"10.1093\/hmg\/ddl434","article-title":"Mutant huntingtin inhibits clathrin-independent endocytosis and causes accumulation of cholesterol in vitro and in vivo","volume":"15","author":"Trushina","year":"2006","journal-title":"Hum Mol Genet"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB56|cit56","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1016\/S1096-7192(02)00010-0","article-title":"Cholesterol storage defect in RSH\/Smith-Lemli-Opitz syndrome fibroblasts","volume":"75","author":"Wassif","year":"2002","journal-title":"Mol Genet Metab"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB57|cit57","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1006\/mgme.2000.3061","article-title":"The structure and function of the Niemann-Pick C1 protein","volume":"71","author":"Ioannou","year":"2000","journal-title":"Mol Genet Metab"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB58|cit58","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1016\/j.bbalip.2004.08.006","article-title":"The NPC1 protein: structure implies function","volume":"1685","author":"Scott","year":"2004","journal-title":"Biochim Biophys Acta"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB59|cit59","first-page":"107","article-title":"The RND permease superfamily: an ancient, ubiquitous and diverse family that includes human disease and development proteins","volume":"1","author":"Tseng","year":"1999","journal-title":"J Mol Microbiol Biotechnol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB60|cit60","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1002\/humu.10255","article-title":"Identification of 58 novel mutations in Niemann-Pick disease type C: correlation with biochemical phenotype and importance of PTC1-like domains in NPC1","volume":"22","author":"Park","year":"2003","journal-title":"Hum Mutat"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB61|cit61","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1186\/gb-2008-9-11-241","article-title":"The Hedgehog protein family","volume":"9","author":"Burglin","year":"2008","journal-title":"Genome Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB62|cit62","doi-asserted-by":"crossref","first-page":"547","DOI":"10.1083\/jcb.200310046","article-title":"Mutagenesis of the putative sterol-sensing domain of yeast Niemann Pick C-related protein reveals a primordial role in subcellular sphingolipid distribution","volume":"164","author":"Malathi","year":"2004","journal-title":"J Cell Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB63|cit63","doi-asserted-by":"crossref","first-page":"769","DOI":"10.1016\/j.bbalip.2007.03.003","article-title":"Sterol transport in yeast and the oxysterol binding protein homologue (OSH) family","volume":"1771","author":"Schulz","year":"2007","journal-title":"Biochim Biophys Acta"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB64|cit64","doi-asserted-by":"crossref","first-page":"1283","DOI":"10.1016\/S0960-9822(01)00396-7","article-title":"Critical role for glycosphingolipids in Niemann-Pick disease type C","volume":"11","author":"Zervas","year":"2001","journal-title":"Curr Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB65|cit65","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1146\/annurev.cellbio.21.122303.120013","article-title":"Principles of lysosomal membrane digestion: stimulation of sphingolipid degradation by sphingolipid activator proteins and anionic lysosomal lipids","volume":"21","author":"Kolter","year":"2005","journal-title":"Annu Rev Cell Dev Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB66|cit66","doi-asserted-by":"crossref","first-page":"12391","DOI":"10.1073\/pnas.221181998","article-title":"Accumulation of cholera toxin and GM1 ganglioside in the early endosome of Niemann-Pick C1-deficient cells","volume":"98","author":"Sugimoto","year":"2001","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB67|cit67","doi-asserted-by":"crossref","first-page":"1087","DOI":"10.1093\/hmg\/9.7.1087","article-title":"Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse","volume":"9","author":"Liu","year":"2000","journal-title":"Hum Mol Genet"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB68|cit68","doi-asserted-by":"crossref","first-page":"1324","DOI":"10.1016\/S0960-9822(03)00531-1","article-title":"Cholesterol accumulation in NPC1-deficient neurons is ganglioside dependent","volume":"13","author":"Gondre-Lewis","year":"2003","journal-title":"Curr Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB69|cit69","doi-asserted-by":"crossref","first-page":"19365","DOI":"10.1074\/jbc.272.31.19365","article-title":"Extensive glycosphingolipid depletion in the liver and lymphoid organs of mice treated with N-butyldeoxynojirimycin","volume":"272","author":"Platt","year":"1997","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB70|cit70","doi-asserted-by":"crossref","first-page":"1619","DOI":"10.1111\/j.1471-4159.2005.03534.x","article-title":"Defective calcium homeostasis in the cerebellum in a mouse model of Niemann-Pick A disease","volume":"95","author":"Ginzburg","year":"2005","journal-title":"J Neurochem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB71|cit71","doi-asserted-by":"crossref","first-page":"113","DOI":"10.1016\/j.ymgme.2005.07.025","article-title":"Niemann-Pick type C disease: novel NPC1 mutations and characterization of the concomitant acid sphingomyelinase deficiency","volume":"87","author":"Tamura","year":"2006","journal-title":"Mol Genet Metab"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB72|cit72","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/S0065-1281(85)80054-4","article-title":"Adrenal changes in Niemann-Pick disease: differences between sphingomyelinase deficiency and type C","volume":"76","author":"Elleder","year":"1985","journal-title":"Acta Histochem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB73|cit73","doi-asserted-by":"crossref","first-page":"38104","DOI":"10.1074\/jbc.M005296200","article-title":"Posttranslational regulation of acid sphingomyelinase in niemann-pick type C1 fibroblasts and free cholesterol-enriched chinese hamster ovary cells","volume":"275","author":"Reagan","year":"2000","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB74|cit74","doi-asserted-by":"crossref","first-page":"11549","DOI":"10.1074\/jbc.M609424200","article-title":"Activation of acid sphingomyelinase by protein kinase Cdelta-mediated phosphorylation","volume":"282","author":"Zeidan","year":"2007","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB75|cit75","doi-asserted-by":"crossref","first-page":"787","DOI":"10.1042\/bj3250787","article-title":"Modulation of protein kinase C by endogenous sphingosine: inhibition of phorbol dibutyrate binding in Niemann-Pick C fibroblasts","volume":"325","author":"Rodriguez-Lafrasse","year":"1997","journal-title":"Biochem J"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB76|cit76","doi-asserted-by":"crossref","first-page":"1154","DOI":"10.1046\/j.1471-4159.2002.01220.x","article-title":"Cholesterol accumulates in cell bodies, but is decreased in distal axons, of Niemann-Pick C1-deficient neurons","volume":"83","author":"Karten","year":"2002","journal-title":"J Neurochem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB77|cit77","doi-asserted-by":"crossref","first-page":"2295","DOI":"10.1126\/science.290.5500.2295","article-title":"Transmembrane molecular pump activity of Niemann-Pick C1 protein","volume":"290","author":"Davies","year":"2000","journal-title":"Science"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB78|cit78","doi-asserted-by":"crossref","first-page":"10333","DOI":"10.1074\/jbc.M413657200","article-title":"Flux of fatty acids through NPC1 lysosomes","volume":"280","author":"Passeggio","year":"2005","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB79|cit79","doi-asserted-by":"crossref","first-page":"24584","DOI":"10.1074\/jbc.M803715200","article-title":"Niemann-Pick C1 functions in regulating lysosomal amine content","volume":"283","author":"Kaufmann","year":"2008","journal-title":"J Biol Chem"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB80|cit80","doi-asserted-by":"crossref","first-page":"403","DOI":"10.1007\/978-1-4615-3362-7_55","article-title":"Sphingosine inhibits calcium release from sarcoplasmic reticulum membranes","volume":"311","author":"Betto","year":"1992","journal-title":"Adv Exp Med Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB81|cit81","doi-asserted-by":"crossref","first-page":"102","DOI":"10.1016\/0006-291X(89)91688-4","article-title":"Inhibition of protein kinase C by sphingosine correlates with the presence of positive charge","volume":"164","author":"Bottega","year":"1989","journal-title":"Biochem Biophys Res Commun"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB82|cit82","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1042\/bj3590335","article-title":"Sphingosine-induced apoptosis is dependent on lysosomal proteases","volume":"359","author":"Kagedal","year":"2001","journal-title":"Biochem J"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB83|cit83","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1042\/bj20030613","article-title":"Lyso-glycosphingolipids mobilize calcium from brain microsomes via multiple mechanisms","volume":"375","author":"Lloyd-Evans","year":"2003","journal-title":"Biochem J"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB84|cit84","doi-asserted-by":"crossref","first-page":"G947","DOI":"10.1152\/ajpgi.00151.2002","article-title":"Tumor necrosis factor-alpha-associated lysosomal permeabilization is cathepsin B dependent","volume":"283","author":"Werneburg","year":"2002","journal-title":"Am J Physiol Gastrointest Liver Physiol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB85|cit85","doi-asserted-by":"crossref","first-page":"139","DOI":"10.1111\/j.1749-6632.1998.tb09667.x","article-title":"Sphingolipid metabolism. Sphingoid analogs, sphingolipid activator proteins, and the pathology of the cell.","volume":"845","author":"Sandhoff","year":"1998","journal-title":"Ann N Y Acad Sci"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB86|cit86","doi-asserted-by":"crossref","first-page":"423","DOI":"10.1016\/S0300-9084(03)00047-6","article-title":"Salvage pathways in glycosphingolipid metabolism","volume":"85","author":"Tettamanti","year":"2003","journal-title":"Biochimie"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB87|cit87","doi-asserted-by":"crossref","first-page":"315","DOI":"10.1159\/000112179","article-title":"Type C Niemann-Pick disease: use of hydrophobic amines to study defective cholesterol transport","volume":"13","author":"Roff","year":"1991","journal-title":"Dev Neurosci"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB88|cit88","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1006\/excr.1997.3612","article-title":"Internalized plasma membrane cholesterol passes through an endosome compartment that is distinct from the acid vesicle-lysosome compartment","volume":"234","author":"Porpaczy","year":"1997","journal-title":"Exp Cell Res"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB89|cit89","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1016\/S0960-9822(07)00565-9","article-title":"Calcium uptake via endocytosis with rapid release from acidifying endosomes","volume":"8","author":"Gerasimenko","year":"1998","journal-title":"Curr Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB90|cit90","doi-asserted-by":"crossref","first-page":"599","DOI":"10.1242\/jcs.115.3.599","article-title":"pH-dependent regulation of lysosomal calcium in macrophages","volume":"115","author":"Christensen","year":"2002","journal-title":"J Cell Sci"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB91|cit91","doi-asserted-by":"crossref","first-page":"703","DOI":"10.1016\/S0092-8674(02)01082-6","article-title":"NAADP mobilizes Ca(2+) from reserve granules, lysosome-related organelles, in sea urchin eggs","volume":"111","author":"Churchill","year":"2002","journal-title":"Cell"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB92|cit92","doi-asserted-by":"crossref","first-page":"531","DOI":"10.1016\/j.ceca.2007.08.006","article-title":"Cell-permeant NAADP: a novel chemical tool enabling the study of Ca2+ signalling in intact cells","volume":"43","author":"Parkesh","year":"2008","journal-title":"Cell Calcium"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB93|cit93","doi-asserted-by":"crossref","first-page":"596","DOI":"10.1038\/nature08030","article-title":"NAADP mobilizes calcium from acidic organelles through two-pore channels","volume":"459","author":"Calcraft","year":"2009","journal-title":"Nature"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB94|cit94","doi-asserted-by":"crossref","first-page":"1053","DOI":"10.1083\/jcb.149.5.1053","article-title":"The role of intraorganellar Ca(2+) in late endosome-lysosome heterotypic fusion and in the reformation of lysosomes from hybrid organelles","volume":"149","author":"Pryor","year":"2000","journal-title":"J Cell Biol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB95|cit95","first-page":"278","article-title":"Loss of membrane cholesterol affects lysosomal osmotic stability","volume":"27","author":"Hao","year":"2008","journal-title":"Gen Physiol Biophys"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB96|cit96","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1097\/00005792-195802000-00001","article-title":"Niemann-Pick disease: a review of eighteen patients","volume":"37","author":"Crocker","year":"1958","journal-title":"Medicine (Baltimore)"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB97|cit97","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1073\/pnas.55.2.366","article-title":"The metabolism of sphingomyelin. II. Evidence of an enzymatic deficiency in Niemann-Pick diseae.","volume":"55","author":"Brady","year":"1966","journal-title":"Proc Natl Acad Sci U S A"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB98|cit98","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1007\/BF00695084","article-title":"Niemann-Pick disease type C with enhanced glycolipid storage. Report on further case of so-called lactosylceramidosis.","volume":"402","author":"Elleder","year":"1984","journal-title":"Virchows Arch A Pathol Anat Histopathol"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB99|cit99","first-page":"274","article-title":"Detection of glycosphingolipids in small samples of human tissue","volume":"2","author":"Dawson","year":"1972","journal-title":"Ann Clin Lab Sci"},{"key":"10.1111\/j.1600-0854.2010.01032.x-BIB100|cit100","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":"10.1111\/j.1600-0854.2010.01032.x-BIB101|cit101","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1210\/en.2005-1091","article-title":"Cyclic adenosine 5\u2032-monophosphate-dependent sphingosine-1-phosphate biosynthesis induces human CYP17 gene transcription by activating cleavage of sterol regulatory element binding protein 1","volume":"147","author":"Ozbay","year":"2006","journal-title":"Endocrinology"}],"container-title":["Traffic"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fj.1600-0854.2010.01032.x","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/onlinelibrary.wiley.com\/wol1\/doi\/10.1111\/j.1600-0854.2010.01032.x\/fullpdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,10,19]],"date-time":"2021-10-19T18:01:47Z","timestamp":1634666507000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/j.1600-0854.2010.01032.x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,3,10]]},"references-count":101,"journal-issue":{"issue":"4"},"URL":"https:\/\/doi.org\/10.1111\/j.1600-0854.2010.01032.x","relation":{},"ISSN":["1398-9219","1600-0854"],"issn-type":[{"value":"1398-9219","type":"print"},{"value":"1600-0854","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,3,10]]}}}