{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,15]],"date-time":"2026-04-15T06:16:09Z","timestamp":1776233769901,"version":"3.50.1"},"reference-count":46,"publisher":"Public Library of Science (PLoS)","issue":"7","license":[{"start":{"date-parts":[[2013,7,2]],"date-time":"2013-07-02T00:00:00Z","timestamp":1372723200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0067084","type":"journal-article","created":{"date-parts":[[2013,7,2]],"date-time":"2013-07-02T21:14:16Z","timestamp":1372799656000},"page":"e67084","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":36,"title":["Pulmonary Abnormalities in Animal Models Due to Niemann-Pick Type C1 (NPC1) or C2 (NPC2) Disease"],"prefix":"10.1371","volume":"8","author":[{"given":"Blair R.","family":"Roszell","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jian-Qin","family":"Tao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kevin J.","family":"Yu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ling","family":"Gao","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shaohui","family":"Huang","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yue","family":"Ning","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sheldon I.","family":"Feinstein","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Charles H.","family":"Vite","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sandra R.","family":"Bates","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"340","published-online":{"date-parts":[[2013,7,2]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"789","DOI":"10.1111\/j.1471-4159.2010.06976.x","article-title":"Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches","volume":"116","author":"AI Rosenbaum","year":"2011","journal-title":"Journal of neurochemistry"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1111\/j.1399-0004.2009.01325.x","article-title":"Respiratory disease in Niemann-Pick type C2 is caused by pulmonary alveolar proteinosis","volume":"77","author":"M Griese","year":"2010","journal-title":"Clin Genet"},{"key":"ref3","first-page":"343","article-title":"Clinical-biochemical correlation in molecularly characterized patients with Niemann-Pick type C. Genetics in medicine : official journal of the American College of Medical Genetics","volume":"3","author":"V Meiner","year":"2001"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1086\/324068","article-title":"Niemann-Pick disease type C: spectrum of HE1 mutations and genotype\/phenotype correlations in the NPC2 group","volume":"69","author":"G Millat","year":"2001","journal-title":"Am J Hum Genet"},{"key":"ref5","first-page":"16","article-title":"Niemann-Pick disease type C. Orphanet journal of rare diseases","volume":"5","author":"MT Vanier","year":"2010"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1007\/BF00203741","article-title":"Ultrastructural changes in the lung in Niemann-Pick type C mouse","volume":"427","author":"T Manabe","year":"1995","journal-title":"Virchows Archiv : an international journal of pathology"},{"key":"ref7","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":"B Liu","year":"2010","journal-title":"J Lipid Res"},{"key":"ref8","first-page":"140","article-title":"Lysosome lipid storage disorder in NCTR-BALB\/c mice. I. Description of the disease and genetics","volume":"108","author":"MD Morris","year":"1982","journal-title":"Am J Pathol"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1016\/S1096-7192(03)00074-X","article-title":"Mutation analysis of feline Niemann-Pick C1 disease","volume":"79","author":"KL Somers","year":"2003","journal-title":"Mol Genet Metab"},{"key":"ref10","first-page":"1412","article-title":"Feline Niemann-Pick disease type C. Am J Pathol","volume":"144","author":"DE Brown","year":"1994"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1152\/ajplegacy.1972.223.3.715","article-title":"Surface active materials from dog lung. II. Composition and physiological correlations","volume":"223","author":"RJ King","year":"1972","journal-title":"American Journal of Physiology"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"90","DOI":"10.1016\/S0925-4439(98)00061-1","article-title":"The role of lipids in pulmonary surfactant","volume":"1408","author":"R Veldhuizen","year":"1998","journal-title":"Biochim Biophys Acta"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1016\/j.bbalip.2005.09.002","article-title":"Pulmonary surfactant function is abolished by an elevated proportion of cholesterol","volume":"1737","author":"L Gunasekara","year":"2005","journal-title":"Biochimica et biophysica acta"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"L117","DOI":"10.1152\/ajplung.00218.2009","article-title":"Role of cholesterol in the biophysical dysfunction of surfactant in ventilator-induced lung injury","volume":"298","author":"D Vockeroth","year":"2010","journal-title":"American journal of physiology Lung cellular and molecular physiology"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"674","DOI":"10.1529\/biophysj.107.106310","article-title":"An elevated level of cholesterol impairs self-assembly of pulmonary surfactant into a functional film","volume":"93","author":"Z Leonenko","year":"2007","journal-title":"Biophysical journal"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"166","DOI":"10.1016\/0005-2760(79)90183-8","article-title":"Surfactant cholesterol metabolism of the isolated perfused rat lung","volume":"573","author":"MA Hass","year":"1979","journal-title":"Biochim Biophys Acta"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"228","DOI":"10.1126\/science.277.5323.228","article-title":"Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis","volume":"277","author":"ED Carstea","year":"1997","journal-title":"Science"},{"key":"ref18","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-Pick C disease","volume":"290","author":"S Naureckiene","year":"2000","journal-title":"Science"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"L919","DOI":"10.1152\/ajplung.00383.2011","article-title":"Characterization of the Niemann-Pick C pathway in alveolar type II cells and lamellar bodies of the lung","volume":"302","author":"BR Roszell","year":"2012","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"15287","DOI":"10.1073\/pnas.0807328105","article-title":"NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes","volume":"105","author":"RE Infante","year":"2008","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref21","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":"MS Deffieu","year":"2011","journal-title":"Proceedings of the National Academy of Sciences of the United States of America"},{"key":"ref22","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":"HJ Kwon","year":"2009","journal-title":"Cell"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1083\/jcb.201004142","article-title":"A role for oxysterol-binding protein-related protein 5 in endosomal cholesterol trafficking","volume":"192","author":"X Du","year":"2011","journal-title":"J Cell Biol"},{"key":"ref24","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":"CM Ramirez","year":"2010","journal-title":"Pediatr Res"},{"key":"ref25","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":"SK Loftus","year":"1997","journal-title":"Science"},{"key":"ref26","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":"DE Sleat","year":"2004","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1203\/PDR.0b013e3181df4623","article-title":"2-hydroxypropyl-beta-cyclodextrin raises hearing threshold in normal cats and in cats with Niemann-Pick type C disease","volume":"68","author":"S Ward","year":"2010","journal-title":"Pediatr Res"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"L869","DOI":"10.1152\/ajplung.00077.2003","article-title":"Identification and characterization of rodent ABCA1 in isolated type II pneumocytes","volume":"285","author":"AE Bortnick","year":"2003","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"ref29","doi-asserted-by":"crossref","first-page":"6126","DOI":"10.1016\/S0021-9258(17)38501-0","article-title":"Lung lamellar bodies maintain an acidic internal pH","volume":"261","author":"A Chander","year":"1986","journal-title":"The Journal of biological chemistry"},{"key":"ref30","doi-asserted-by":"crossref","first-page":"265","DOI":"10.1016\/S0021-9258(19)52451-6","article-title":"Protein measurement with the Folin phenol reagent","volume":"193","author":"OH Lowry","year":"1951","journal-title":"J Biol Chem"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1016\/S0021-9258(18)70226-3","article-title":"Phosphorus assay in column chromatography","volume":"234","author":"GR Bartlett","year":"1959","journal-title":"J Biol Chem"},{"key":"ref32","first-page":"911","article-title":"A rapid method of total lipid extraction and purification","volume":"37","author":"EG Bligh","year":"1959","journal-title":"Can J Med Sci"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"L759","DOI":"10.1152\/ajplung.00200.2002","article-title":"SP-A is necessary for increased clearance of alveolar DPPC with hyperventilation or secretagogues","volume":"284","author":"D Jain","year":"2003","journal-title":"American journal of physiology Lung cellular and molecular physiology"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1016\/S0022-2275(20)36986-8","article-title":"Isolation of disaturated phosphatidylcholine with osmium tetroxide","volume":"17","author":"RJ Mason","year":"1976","journal-title":"Journal of lipid research"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"2848","DOI":"10.1002\/jnr.21730","article-title":"Astrocyte-only Npc1 reduces neuronal cholesterol and triples life span of Npc1\u2212\/\u2212 mice","volume":"86","author":"M Zhang","year":"2008","journal-title":"Journal of neuroscience research"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"L1266","DOI":"10.1152\/ajplung.2001.280.6.L1266","article-title":"Macrophage and type II cell catabolism of SP-A and saturated phosphatidylcholine in mouse lungs","volume":"280","author":"O Gurel","year":"2001","journal-title":"American journal of physiology Lung cellular and molecular physiology"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1016\/j.ymgme.2011.03.001","article-title":"Pulmonary function and pathology in hydroxypropyl-beta-cyclodextin-treated and untreated Npc1\/mice","volume":"103","author":"A Muralidhar","year":"2011","journal-title":"Mol Genet Metab"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1093\/hmg\/ddr505","article-title":"A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations","volume":"21","author":"RA Maue","year":"2012","journal-title":"Hum Mol Genet"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1165\/rcmb.2004-0293OC","article-title":"Defective surfactant secretion in a mouse model of Hermansky-Pudlak syndrome","volume":"33","author":"SH Guttentag","year":"2005","journal-title":"Am J Respir Cell Mol Biol"},{"key":"ref40","unstructured":"Fisher AB (1998) Lung Surfactant Clearance and Cellular Processing. In: Rooney SA, Landes RG, editors. Lung surfactant: cellular and molecular processing. Austin, TX: Landes Bioscience. 165\u2013189."},{"key":"ref41","doi-asserted-by":"crossref","first-page":"L646","DOI":"10.1152\/ajplung.00409.2009","article-title":"Conditional deletion of Abca3 in alveolar type II cells alters surfactant homeostasis in newborn and adult mice","volume":"298","author":"V Besnard","year":"2010","journal-title":"American journal of physiology Lung cellular and molecular physiology"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1007\/s004280000241","article-title":"Interstitial pneumonia in Hermansky-Pudlak syndrome: significance of florid foamy swelling\/degeneration (giant lamellar body degeneration) of type-2 pneumocytes","volume":"437","author":"Y Nakatani","year":"2000","journal-title":"Virchows Archiv : an international journal of pathology"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/S1084952102000551","article-title":"Biogenesis of lamellar bodies, lysosome-related organelles involved in storage and secretion of pulmonary surfactant","volume":"13","author":"TE Weaver","year":"2002","journal-title":"Semin Cell Dev Biol"},{"key":"ref44","doi-asserted-by":"crossref","first-page":"1747","DOI":"10.1194\/jlr.M003822","article-title":"Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion","volume":"51","author":"M Abdul-Hammed","year":"2010","journal-title":"J Lipid Res"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1016\/S1095-6433(01)00307-5","article-title":"The roles of cholesterol in pulmonary surfactant: insights from comparative and evolutionary studies","volume":"129","author":"S Orgeig","year":"2001","journal-title":"Comp Biochem Physiol A Mol Integr Physiol"},{"key":"ref46","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1016\/j.abb.2011.07.014","article-title":"Free cholesterol accumulation impairs antioxidant activities and aggravates apoptotic cell death in menadione-induced oxidative injury","volume":"514","author":"W Lee","year":"2011","journal-title":"Archives of biochemistry and biophysics"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0067084","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,2,27]],"date-time":"2022-02-27T02:26:55Z","timestamp":1645928815000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0067084"}},"subtitle":[],"editor":[{"given":"Emanuele","family":"Buratti","sequence":"first","affiliation":[],"role":[{"role":"editor","vocabulary":"crossref"}]}],"short-title":[],"issued":{"date-parts":[[2013,7,2]]},"references-count":46,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2013,7,2]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0067084","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,7,2]]}}}