{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T09:19:59Z","timestamp":1783415999530,"version":"3.54.6"},"reference-count":31,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2019,3,1]],"date-time":"2019-03-01T00:00:00Z","timestamp":1551398400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2018,6,15]],"date-time":"2018-06-15T00:00:00Z","timestamp":1529020800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["R01EB009481"],"award-info":[{"award-number":["R01EB009481"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"NIH","doi-asserted-by":"publisher","award":["2P30DK027651"],"award-info":[{"award-number":["2P30DK027651"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"name":"CFF RDP","award":["R447-CR11"],"award-info":[{"award-number":["R447-CR11"]}]}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","cysticfibrosisjournal.com","clinicalkey.es","clinicalkey.fr","clinicalkey.com.au","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Journal of Cystic Fibrosis"],"published-print":{"date-parts":[[2019,3]]},"DOI":"10.1016\/j.jcf.2018.06.005","type":"journal-article","created":{"date-parts":[[2018,6,22]],"date-time":"2018-06-22T10:34:42Z","timestamp":1529663682000},"page":"175-181","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":10,"title":["Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent"],"prefix":"10.1016","volume":"18","author":[{"given":"Binyu","family":"Lu","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Li","family":"Li","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Molly","family":"Schneider","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Craig A.","family":"Hodges","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Calvin U.","family":"Cotton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James D.","family":"Burgess","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas J.","family":"Kelley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"4792","key":"10.1016\/j.jcf.2018.06.005_bb0005","doi-asserted-by":"crossref","first-page":"1046","DOI":"10.1126\/science.2950591","article-title":"Construction of a general human chromosome jumping library, with application to cystic fibrosis","volume":"235","author":"Collins","year":"1987","journal-title":"Science (New York, NY)"},{"issue":"6","key":"10.1016\/j.jcf.2018.06.005_bb0010","doi-asserted-by":"crossref","first-page":"1227","DOI":"10.1016\/0092-8674(90)90398-X","article-title":"Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer","volume":"62","author":"Drumm","year":"1990","journal-title":"Cell"},{"issue":"5","key":"10.1016\/j.jcf.2018.06.005_bb0015","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"},{"issue":"2","key":"10.1016\/j.jcf.2018.06.005_bb0020","doi-asserted-by":"crossref","first-page":"L476","DOI":"10.1152\/ajplung.00262.2006","article-title":"Altered cholesterol homeostasis in cultured and in vivo models of cystic fibrosis","volume":"292","author":"White","year":"2007","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"6","key":"10.1016\/j.jcf.2018.06.005_bb0025","doi-asserted-by":"crossref","first-page":"L419","DOI":"10.1152\/ajplung.00411.2012","article-title":"Reduced microtubule acetylation in cystic fibrosis epithelial cells","volume":"305","author":"Rymut","year":"2013","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"6","key":"10.1016\/j.jcf.2018.06.005_bb0030","doi-asserted-by":"crossref","first-page":"853","DOI":"10.1165\/rcmb.2014-0462OC","article-title":"Role of exchange protein activated by cAMP 1 in regulating rates of microtubule formation in cystic fibrosis epithelial cells","volume":"53","author":"Rymut","year":"2015","journal-title":"Am J Respir Cell Mol Biol"},{"issue":"6887","key":"10.1016\/j.jcf.2018.06.005_bb0035","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1038\/417455a","article-title":"HDAC6 is a microtubule-associated deacetylase","volume":"417","author":"Hubbert","year":"2002","journal-title":"Nature"},{"key":"10.1016\/j.jcf.2018.06.005_bb0040","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1186\/1465-9921-11-61","article-title":"Increased plasma membrane cholesterol in cystic fibrosis cells correlates with CFTR genotype and depends on de novo cholesterol synthesis","volume":"11","author":"Fang","year":"2010","journal-title":"Respir Res"},{"issue":"37","key":"10.1016\/j.jcf.2018.06.005_bb0045","doi-asserted-by":"crossref","first-page":"11352","DOI":"10.1021\/ja074373c","article-title":"Direct electrochemical evaluation of plasma membrane cholesterol in live mammalian cells","volume":"129","author":"Jiang","year":"2007","journal-title":"J Am Chem Soc"},{"issue":"6","key":"10.1016\/j.jcf.2018.06.005_bb0050","doi-asserted-by":"crossref","DOI":"10.1149\/2.005406jes","article-title":"Double potential pulse chronocoulometry for detection of plasma membrane cholesterol efflux at disk platinum microelectrodes","volume":"161","author":"West","year":"2014","journal-title":"J Electrochem Soc"},{"issue":"11","key":"10.1016\/j.jcf.2018.06.005_bb0055","doi-asserted-by":"crossref","first-page":"1433","DOI":"10.1164\/rccm.201710-1983OC","article-title":"Effects of Lumacaftor\/Ivacaftor therapy on CFTR function in Phe508del homozygous patients with cystic fibrosis","volume":"197","author":"Graeber","year":"2018","journal-title":"Am J Respir Crit Care Med"},{"issue":"18","key":"10.1016\/j.jcf.2018.06.005_bb0060","doi-asserted-by":"crossref","first-page":"1663","DOI":"10.1056\/NEJMoa1105185","article-title":"A CFTR potentiator in patients with cystic fibrosis and the G551D mutation","volume":"365","author":"Ramsey","year":"2011","journal-title":"N Engl J Med"},{"issue":"15","key":"10.1016\/j.jcf.2018.06.005_bb0065","doi-asserted-by":"crossref","first-page":"2037","DOI":"10.1042\/CS20170995","article-title":"Improvement in exercise duration, lung function and well-being in G551D-cystic fibrosis patients: A double-blind, placebo-controlled, randomized, cross-over study with ivacaftor treatment","volume":"131","author":"Edgeworth","year":"2017","journal-title":"Clin Sci (London, England: 1979)"},{"issue":"1","key":"10.1016\/j.jcf.2018.06.005_bb0070","doi-asserted-by":"crossref","first-page":"83","DOI":"10.1016\/j.jcf.2017.06.002","article-title":"Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor","volume":"17","author":"Flume","year":"2018","journal-title":"J Cyst Fibros"},{"issue":"2","key":"10.1016\/j.jcf.2018.06.005_bb0075","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1016\/j.chest.2017.10.005","article-title":"CORK study in cystic fibrosis: Sustained improvements in ultra-low-dose chest CT scores after CFTR modulation with Ivacaftor","volume":"153","author":"Ronan","year":"2017","journal-title":"Chest"},{"issue":"11","key":"10.1016\/j.jcf.2018.06.005_bb0080","doi-asserted-by":"crossref","first-page":"2053","DOI":"10.4161\/15548627.2014.973737","article-title":"Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation","volume":"10","author":"De Stefano","year":"2014","journal-title":"Autophagy"},{"issue":"6","key":"10.1016\/j.jcf.2018.06.005_bb0085","doi-asserted-by":"crossref","first-page":"714","DOI":"10.1016\/j.jcf.2016.10.009","article-title":"Biomarkers for cystic fibrosis drug development","volume":"15","author":"Muhlebach","year":"2016","journal-title":"J Cyst Fibros"},{"issue":"2","key":"10.1016\/j.jcf.2018.06.005_bb0090","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1002\/ppul.10043","article-title":"Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis","volume":"33","author":"Ahrens","year":"2002","journal-title":"Pediatr Pulmonol"},{"issue":"21","key":"10.1016\/j.jcf.2018.06.005_bb0095","doi-asserted-by":"crossref","first-page":"1991","DOI":"10.1056\/NEJMoa0909825","article-title":"Effect of VX-770 in persons with cystic fibrosis and the G551D-CFTR mutation","volume":"363","author":"Accurso","year":"2010","journal-title":"N Engl J Med"},{"issue":"3","key":"10.1016\/j.jcf.2018.06.005_bb0100","doi-asserted-by":"crossref","first-page":"G528","DOI":"10.1152\/ajpgi.00052.2011","article-title":"Cystic fibrosis growth retardation is not correlated with loss of Cftr in the intestinal epithelium","volume":"301","author":"Hodges","year":"2011","journal-title":"Am J Physiol Gastrointest Liver Physiol"},{"issue":"7128","key":"10.1016\/j.jcf.2018.06.005_bb0105","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1038\/nature05541","article-title":"Restoration of p53 function leads to tumour regression in vivo","volume":"445","author":"Ventura","year":"2007","journal-title":"Nature"},{"issue":"5","key":"10.1016\/j.jcf.2018.06.005_bb0110","doi-asserted-by":"crossref","first-page":"L1173","DOI":"10.1152\/ajplung.2001.281.5.L1173","article-title":"Examining basal chloride transport using the nasal potential difference response in a murine model","volume":"281","author":"Brady","year":"2001","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"5 Pt 1","key":"10.1016\/j.jcf.2018.06.005_bb0115","first-page":"L1065","article-title":"In vivo activation of CFTR-dependent chloride transport in murine airway epithelium by CNP","volume":"273","author":"Kelley","year":"1997","journal-title":"Am J Physiol"},{"issue":"6 Pt 1","key":"10.1016\/j.jcf.2018.06.005_bb0120","first-page":"L990","article-title":"Regulation of amiloride-sensitive sodium absorption in murine airway epithelium by C-type natriuretic peptide","volume":"274","author":"Kelley","year":"1998","journal-title":"Am J Physiol"},{"issue":"6","key":"10.1016\/j.jcf.2018.06.005_bb0125","doi-asserted-by":"crossref","first-page":"2604","DOI":"10.1073\/pnas.94.6.2604","article-title":"In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium","volume":"94","author":"Kelley","year":"1997","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"4","key":"10.1016\/j.jcf.2018.06.005_bb0130","doi-asserted-by":"crossref","first-page":"1235","DOI":"10.1021\/ac7019909","article-title":"Electrochemical analysis of cell plasma membrane cholesterol at the airway surface of mouse trachea","volume":"80","author":"Jiang","year":"2008","journal-title":"Anal Chem"},{"issue":"1","key":"10.1016\/j.jcf.2018.06.005_bb0135","doi-asserted-by":"crossref","first-page":"3676","DOI":"10.1038\/s41598-017-03931-2","article-title":"Improved growth patterns in cystic fibrosis mice after loss of histone deacetylase 6","volume":"7","author":"Rymut","year":"2017","journal-title":"Sci Rep"},{"issue":"8","key":"10.1016\/j.jcf.2018.06.005_bb0140","doi-asserted-by":"crossref","DOI":"10.1149\/2.1001608jes","article-title":"Communication-microelectrode detection of cholesterol efflux from the human buccel mucosa","volume":"163","author":"Yu","year":"2016","journal-title":"J Electrochem Soc"},{"issue":"5","key":"10.1016\/j.jcf.2018.06.005_bb0145","doi-asserted-by":"crossref","first-page":"L809","DOI":"10.1152\/ajplung.90402.2008","article-title":"cAMP-mediated regulation of cholesterol accumulation in cystic fibrosis and Niemann-pick type C cells","volume":"295","author":"Manson","year":"2008","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"issue":"1","key":"10.1016\/j.jcf.2018.06.005_bb0150","doi-asserted-by":"crossref","first-page":"2","DOI":"10.1186\/s12931-017-0705-8","article-title":"Inhibition of histone-deacetylase activity rescues inflammatory cystic fibrosis lung disease by modulating innate and adaptive immune responses","volume":"19","author":"Bodas","year":"2018","journal-title":"Respir Res"},{"issue":"34","key":"10.1016\/j.jcf.2018.06.005_bb0155","doi-asserted-by":"crossref","first-page":"12038","DOI":"10.1021\/ja903684f","article-title":"Observation of cellular cholesterol efflux at microcavity electrodes","volume":"131","author":"Fang","year":"2009","journal-title":"J Am Chem Soc"}],"container-title":["Journal of Cystic Fibrosis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199318306301?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1569199318306301?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2019,8,8]],"date-time":"2019-08-08T18:37:24Z","timestamp":1565289444000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1569199318306301"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,3]]},"references-count":31,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2019,3]]}},"alternative-id":["S1569199318306301"],"URL":"https:\/\/doi.org\/10.1016\/j.jcf.2018.06.005","relation":{},"ISSN":["1569-1993"],"issn-type":[{"value":"1569-1993","type":"print"}],"subject":[],"published":{"date-parts":[[2019,3]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Electrochemical measurement of membrane cholesterol correlates with CFTR function and is HDAC6-dependent","name":"articletitle","label":"Article Title"},{"value":"Journal of Cystic Fibrosis","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jcf.2018.06.005","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2018 The Author(s). Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society.","name":"copyright","label":"Copyright"}]}}