{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T10:48:54Z","timestamp":1788000534460,"version":"build-2784847793"},"reference-count":51,"publisher":"Elsevier BV","issue":"11","license":[{"start":{"date-parts":[[2012,11,1]],"date-time":"2012-11-01T00:00:00Z","timestamp":1351728000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,10,2]],"date-time":"2020-10-02T00:00:00Z","timestamp":1601596800000},"content-version":"vor","delay-in-days":2892,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal of Lipid Research"],"published-print":{"date-parts":[[2012,11]]},"DOI":"10.1194\/jlr.m028241","type":"journal-article","created":{"date-parts":[[2012,8,15]],"date-time":"2012-08-15T04:32:59Z","timestamp":1345005179000},"page":"2331-2342","source":"Crossref","is-referenced-by-count":64,"title":["Cyclodextrin mediates rapid changes in lipid balance in Npc1 mice without carrying cholesterol through the bloodstream"],"prefix":"10.1016","volume":"53","author":[{"given":"Anna M.","family":"Taylor","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bing","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yelenis","family":"Mari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Benny","family":"Liu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joyce J.","family":"Repa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1194\/jlr.M028241_bib1","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1002\/jps.2600810610","article-title":"Hydroxypropylcyclodextrins in parenteral use. II: Effects on transport and disposition of lipids in rabbit and humans","volume":"81","author":"Irie","year":"1992","journal-title":"J. Pharm. Sci."},{"key":"10.1194\/jlr.M028241_bib2","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1615\/CritRevTherDrugCarrierSyst.v14.i1.10","article-title":"Cyclodextrins\u2013enabling excipients: their present and future use in pharmaceuticals","volume":"14","author":"Thompson","year":"1997","journal-title":"Crit. Rev. Ther. Drug Carrier Syst."},{"key":"10.1194\/jlr.M028241_bib3","doi-asserted-by":"crossref","first-page":"30","DOI":"10.1177\/0192623307310945","article-title":"Cyclodextrins","volume":"36","author":"Stella","year":"2008","journal-title":"Toxicol. Pathol."},{"key":"10.1194\/jlr.M028241_bib4","doi-asserted-by":"crossref","first-page":"1451","DOI":"10.1016\/j.fct.2005.03.007","article-title":"2-Hydroxypropyl-\u03b2-cyclodextrin (HP-\u03b2-CD): a toxicology review","volume":"43","author":"Gould","year":"2005","journal-title":"Food Chem. Toxicol."},{"key":"10.1194\/jlr.M028241_bib5","doi-asserted-by":"crossref","first-page":"2377","DOI":"10.1073\/pnas.0810895106","article-title":"Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1\u2212\/\u2212 mouse","volume":"106","author":"Liu","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.M028241_bib6","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":"10.1194\/jlr.M028241_bib7","series-title":"The Metabolic and Molecular Bases of Inherited Disease","first-page":"3611","article-title":"Niemann-Pick Disease Type C: a lipid trafficking disorder","author":"Patterson","year":"2001"},{"key":"10.1194\/jlr.M028241_bib8","doi-asserted-by":"crossref","first-page":"16","DOI":"10.1186\/1750-1172-5-16","article-title":"Niemann-Pick disease type C","volume":"5","author":"Vanier","year":"2010","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1194\/jlr.M028241_bib9","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1194\/jlr.M700525-JLR200","article-title":"Genetic variations and treatments that affect the lifespan of the NPC1 mouse","volume":"49","author":"Liu","year":"2008","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.M028241_bib10","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1203\/PDR.0b013e3181df4623","article-title":"2-Hydroxypropyl-\u03b2-cyclodextrin raises hearing threshold in normal cats and in cats with Niemann-Pick type C disease","volume":"68","author":"Ward","year":"2010","journal-title":"Pediatr. Res."},{"key":"10.1194\/jlr.M028241_bib11","doi-asserted-by":"crossref","first-page":"e6951","DOI":"10.1371\/journal.pone.0006951","article-title":"Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuornal cholesterol and glycosphingolipid storage and disease progression","volume":"4","author":"Davidson","year":"2009","journal-title":"PLoS ONE."},{"key":"10.1194\/jlr.M028241_bib12","doi-asserted-by":"crossref","first-page":"309","DOI":"10.1203\/PDR.0b013e3181ee4dd2","article-title":"Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life","volume":"68","author":"Ramirez","year":"2010","journal-title":"Pediatr. Res."},{"key":"10.1194\/jlr.M028241_bib13","doi-asserted-by":"crossref","first-page":"9404","DOI":"10.1523\/JNEUROSCI.1317-11.2011","article-title":"Unesterified cholesterol accumulation in late endosomes\/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment","volume":"31","author":"Aqul","year":"2011","journal-title":"J. Neurosci."},{"key":"10.1194\/jlr.M028241_bib14","doi-asserted-by":"crossref","first-page":"19316","DOI":"10.1073\/pnas.0910916106","article-title":"Cyclodextrin overcomes deficient lysosome-to-endoplasmic reticulum transport of cholesterol in Niemann-Pick type C cells","volume":"106","author":"Abi-Mosleh","year":"2009","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.M028241_bib15","doi-asserted-by":"crossref","first-page":"5477","DOI":"10.1073\/pnas.0914309107","article-title":"Endocytosis of beta-cyclodextrins is responsible for cholesterol reduction in Niemann-Pick type C mutant cells","volume":"107","author":"Rosenbaum","year":"2010","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.M028241_bib16","doi-asserted-by":"crossref","first-page":"7341","DOI":"10.1021\/bi200574f","article-title":"Sterol transfer between cyclodextrin and membranes: similar but not identical mechanism to NPC2-mediated cholesterol transfer","volume":"50","author":"McCauliff","year":"2011","journal-title":"Biochemistry."},{"key":"10.1194\/jlr.M028241_bib17","doi-asserted-by":"crossref","first-page":"688","DOI":"10.1194\/jlr.M013789","article-title":"Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations","volume":"52","author":"Ramirez","year":"2011","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.M028241_bib18","doi-asserted-by":"crossref","first-page":"933","DOI":"10.1194\/jlr.M000257","article-title":"Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid","volume":"51","author":"Liu","year":"2010","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.M028241_bib19","doi-asserted-by":"crossref","first-page":"232","DOI":"10.1126\/science.277.5323.232","article-title":"Murine model of Niemann-Pick C disease: mutation in a cholesterol homeostasis gene","volume":"277","author":"Loftus","year":"1997","journal-title":"Science."},{"key":"10.1194\/jlr.M028241_bib20","doi-asserted-by":"crossref","first-page":"1469","DOI":"10.1016\/S0022-2275(20)34420-5","article-title":"Measurement of rates of cholesterol synthesis using tritiated water","volume":"25","author":"Dietschy","year":"1984","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.M028241_bib21","doi-asserted-by":"crossref","first-page":"328","DOI":"10.1016\/S0022-2275(20)41221-0","article-title":"Reevaluation and application of the dual-isotope plasma ratio method for the measurement of intestinal cholesterol absorption in the hamster","volume":"35","author":"Turley","year":"1994","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.M028241_bib22","doi-asserted-by":"crossref","first-page":"869","DOI":"10.1194\/jlr.M600488-JLR200","article-title":"Lysosomal unesterifed cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease","volume":"48","author":"Beltroy","year":"2007","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.M028241_bib23","doi-asserted-by":"crossref","first-page":"28103","DOI":"10.1074\/jbc.M504609200","article-title":"Caveolin-1 is not required for murine intestinal cholesterol transport","volume":"280","author":"Valasek","year":"2005","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.M028241_bib24","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/S0076-6879(04)89001-3","article-title":"Quantitative real-time polymerase chain reaction measurement of regulators of G-protein signaling mRNA levels in mouse tissues","volume":"389","author":"Kurrasch","year":"2004","journal-title":"Methods Enzymol."},{"key":"10.1194\/jlr.M028241_bib25","doi-asserted-by":"crossref","first-page":"1101","DOI":"10.1038\/nprot.2008.73","article-title":"Analyzing real-time PCR data by the comparative CT method","volume":"3","author":"Schmittgen","year":"2008","journal-title":"Nat. Protoc."},{"key":"10.1194\/jlr.M028241_bib26","doi-asserted-by":"crossref","first-page":"112","DOI":"10.2144\/04371RR03","article-title":"Validation of housekeeping genes for normalizing RNA expression in real-time PCR","volume":"37","author":"Dheda","year":"2004","journal-title":"Biotechniques."},{"key":"10.1194\/jlr.M028241_bib27","doi-asserted-by":"crossref","first-page":"60","DOI":"10.1186\/1471-2199-11-60","article-title":"Determination of reference genes for circadian studies in different tissues and mouse strains","volume":"11","author":"Kosir","year":"2010","journal-title":"BMC Mol. Biol."},{"key":"10.1194\/jlr.M028241_bib28","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-Pick Type C disease","volume":"64","author":"Li","year":"2005","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1194\/jlr.M028241_bib29","doi-asserted-by":"crossref","first-page":"185","DOI":"10.3109\/10409238.2010.485605","article-title":"Control of cholesterol synthesis through regulated ER-associated degradation of HMG CoA reductase","volume":"45","author":"Jo","year":"2010","journal-title":"Crit. Rev. Biochem. Mol. Biol."},{"key":"10.1194\/jlr.M028241_bib30","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1016\/S0092-8674(02)00722-5","article-title":"Coordinated transcription of key pathways in the mouse by the circadian clock","volume":"109","author":"Panda","year":"2002","journal-title":"Cell."},{"key":"10.1194\/jlr.M028241_bib31","doi-asserted-by":"crossref","first-page":"2061","DOI":"10.1194\/jlr.M500133-JLR200","article-title":"Alternate transcripts expressed in response to diet reflect tissue-specific regulation of ABCA1","volume":"46","author":"Singaraja","year":"2005","journal-title":"J. Lipid Res."},{"key":"10.1194\/jlr.M028241_bib32","doi-asserted-by":"crossref","first-page":"1106","DOI":"10.1093\/jnen\/59.12.1106","article-title":"Cholesterol is sequestered in the brains of mice with Niemann-Pick Type C disease but turnover is increased","volume":"59","author":"Xie","year":"2000","journal-title":"J. Neuropathol. Exp. Neurol."},{"key":"10.1194\/jlr.M028241_bib33","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1016\/j.ymgme.2004.08.017","article-title":"Apoptosis accompanied by up-regulation of TNF-\u03b1 death pathway genes in the brain of Niemann-Pick type C disease","volume":"84","author":"Wu","year":"2005","journal-title":"Mol. Genet. Metab."},{"key":"10.1194\/jlr.M028241_bib34","doi-asserted-by":"crossref","first-page":"13807","DOI":"10.1073\/pnas.0606218103","article-title":"Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease","volume":"103","author":"Langmade","year":"2006","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.M028241_bib35","doi-asserted-by":"crossref","first-page":"14470","DOI":"10.1523\/JNEUROSCI.4823-07.2007","article-title":"Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse","volume":"27","author":"Repa","year":"2007","journal-title":"J. Neurosci."},{"key":"10.1194\/jlr.M028241_bib36","doi-asserted-by":"crossref","first-page":"16026","DOI":"10.1074\/jbc.271.27.16026","article-title":"Cellular cholesterol efflux mediated by cyclodextrins. Demonstration of kinetic pools and mechanism of efflux","volume":"271","author":"Yancey","year":"1996","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.M028241_bib37","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1172\/JCI119223","article-title":"Cyclodextrins as catalysts for the removal of cholesterol from macrophage foam cells","volume":"99","author":"Atger","year":"1997","journal-title":"J. Clin. Invest."},{"key":"10.1194\/jlr.M028241_bib38","doi-asserted-by":"crossref","first-page":"1311","DOI":"10.1016\/j.bbamem.2007.03.026","article-title":"Use of cyclodextrins to manipulate plasma membrane cholesterol content: evidence, misconceptions and control strategies","volume":"1768","author":"Zidovetzki","year":"2007","journal-title":"Biochim. Biophys. Acta."},{"key":"10.1194\/jlr.M028241_bib39","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1006\/jcis.1996.0335","article-title":"Interaction of natural and modified \u03b2-cyclodextrins with a biological membrane model of dipalmitoylphosphatidylcholine","volume":"180","author":"Puglisi","year":"1996","journal-title":"J. Colloid Interface Sci."},{"key":"10.1194\/jlr.M028241_bib40","doi-asserted-by":"crossref","first-page":"e1002020","DOI":"10.1371\/journal.pcbi.1002020","article-title":"Molecular mechanism of cyclodextrin mediated cholesterol extraction","volume":"7","author":"L\u00f3pez","year":"2011","journal-title":"PLOS Comput. Biol."},{"key":"10.1194\/jlr.M028241_bib41","doi-asserted-by":"crossref","first-page":"927","DOI":"10.1002\/jps.2600840805","article-title":"Preliminary safety evaluation of parenterally administered sulfoalkyl ether \u03b2-cyclodextrin derivatives","volume":"84","author":"Rajewski","year":"1995","journal-title":"J. Pharm. Sci."},{"key":"10.1194\/jlr.M028241_bib42","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":"10.1194\/jlr.M028241_bib43","doi-asserted-by":"crossref","first-page":"18932","DOI":"10.1073\/pnas.1110439108","article-title":"Niemann-Pick type C 1 function requires lumenal domain residues that mediate cholesterol-dependent NPC2 binding","volume":"108","author":"Deffieu","year":"2011","journal-title":"Proc. Natl. Acad. Sci. USA."},{"key":"10.1194\/jlr.M028241_bib44","doi-asserted-by":"crossref","first-page":"1465","DOI":"10.1042\/BST0381465","article-title":"Treatments for lysosomal storage disorders","volume":"38","author":"Lachmann","year":"2010","journal-title":"Biochem. Soc. Trans."},{"key":"10.1194\/jlr.M028241_bib45","doi-asserted-by":"crossref","first-page":"1","DOI":"10.2174\/157488911794079127","article-title":"Recent developments in therapeutic approaches for lysosomal storage diseases","volume":"6","author":"Urbanelli","year":"2011","journal-title":"Recent Pat. CNS Drug Discov."},{"key":"10.1194\/jlr.M028241_bib46","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-Pick type C disease","volume":"22","author":"Alvarez","year":"2008","journal-title":"FASEB J."},{"key":"10.1194\/jlr.M028241_bib47","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1016\/j.nbd.2009.07.010","article-title":"Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1","volume":"36","author":"Smith","year":"2009","journal-title":"Neurobiol. Dis."},{"key":"10.1194\/jlr.M028241_bib48","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":"10.1194\/jlr.M028241_bib49","doi-asserted-by":"crossref","first-page":"505","DOI":"10.1016\/j.chemphyslip.2012.03.007","article-title":"Uptake of a fluorescent methyl-\u03b2-cyclodextrin via clathrin-dependent endocytosis","volume":"165","author":"Plazzo","year":"2012","journal-title":"Chem. Phys. Lipids."},{"key":"10.1194\/jlr.M028241_bib50","doi-asserted-by":"crossref","first-page":"9290","DOI":"10.1074\/jbc.M111.326405","article-title":"Normalization of cholesterol homeostasis by 2-hydroxypropyl-\u03b2-cyclodextrin in neurons and glia from Niemann-Pick C1 (NPC1)-deficient mice","volume":"287","author":"Peake","year":"2012","journal-title":"J. Biol. Chem."},{"key":"10.1194\/jlr.M028241_bib51","doi-asserted-by":"crossref","first-page":"833","DOI":"10.1002\/jps.2600830615","article-title":"Parenteral hydroxypropyl cyclodextrins: intravenous and intracerebral administration of lipophiles","volume":"83","author":"Pitha","year":"1994","journal-title":"J. Pharm. Sci."}],"container-title":["Journal of Lipid Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022227520412507?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0022227520412507?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1194\/jlr.M028241","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,11,20]],"date-time":"2021-11-20T22:17:21Z","timestamp":1637446641000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0022227520412507"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,11]]},"references-count":51,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2012,11]]}},"alternative-id":["S0022227520412507"],"URL":"https:\/\/doi.org\/10.1194\/jlr.m028241","relation":{},"ISSN":["0022-2275"],"issn-type":[{"value":"0022-2275","type":"print"}],"subject":[],"published":{"date-parts":[[2012,11]]}}}