{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T20:34:22Z","timestamp":1791232462450,"version":"4.1.0"},"reference-count":50,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2009,12,6]],"date-time":"2009-12-06T00:00:00Z","timestamp":1260057600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Nat Chem Biol"],"published-print":{"date-parts":[[2010,1]]},"DOI":"10.1038\/nchembio.275","type":"journal-article","created":{"date-parts":[[2009,12,7]],"date-time":"2009-12-07T05:19:38Z","timestamp":1260163178000},"page":"25-33","source":"Crossref","is-referenced-by-count":220,"title":["Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis"],"prefix":"10.1038","volume":"6","author":[{"given":"Darren M","family":"Hutt","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Herman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana P C","family":"Rodrigues","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Noel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joseph M","family":"Pilewski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeanne","family":"Matteson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ben","family":"Hoch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy","family":"Kellner","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffery W","family":"Kelly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andre","family":"Schmidt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philip J","family":"Thomas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoshihiro","family":"Matsumura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William R","family":"Skach","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martina","family":"Gentzsch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John R","family":"Riordan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric J","family":"Sorscher","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tsukasa","family":"Okiyoneda","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"III","given":"John R","family":"Yates","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gergely L","family":"Lukacs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymond A","family":"Frizzell","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gerard","family":"Manning","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joel M","family":"Gottesfeld","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William E","family":"Balch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2009,12,6]]},"reference":[{"key":"BFnchembio275_CR1","doi-asserted-by":"publisher","first-page":"701","DOI":"10.1146\/annurev.biochem.75.103004.142532","volume":"77","author":"JR Riordan","year":"2008","unstructured":"Riordan, J.R. CFTR function and prospects for therapy. Annu. Rev. Biochem. 77, 701\u2013726 (2008).","journal-title":"Annu. Rev. Biochem."},{"key":"BFnchembio275_CR2","doi-asserted-by":"publisher","first-page":"15739","DOI":"10.1074\/jbc.272.25.15739","volume":"272","author":"BH Qu","year":"1997","unstructured":"Qu, B.H., Strickland, E.H. & Thomas, P.J. Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding. J. Biol. Chem. 272, 15739\u201315744 (1997).","journal-title":"J. Biol. Chem."},{"key":"BFnchembio275_CR3","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1111\/j.1365-2796.2006.01744.x","volume":"261","author":"RC Boucher","year":"2007","unstructured":"Boucher, R.C. Evidence for airway surface dehydration as the initiating event in CF airway disease. J. Intern. Med. 261, 5\u201316 (2007).","journal-title":"J. Intern. Med."},{"key":"BFnchembio275_CR4","doi-asserted-by":"publisher","first-page":"916","DOI":"10.1126\/science.1141448","volume":"319","author":"WE Balch","year":"2008","unstructured":"Balch, W.E., Morimoto, R.I., Dillin, A. & Kelly, J.W. Adapting proteostasis for disease intervention. Science 319, 916\u2013919 (2008).","journal-title":"Science"},{"key":"BFnchembio275_CR5","doi-asserted-by":"publisher","first-page":"959","DOI":"10.1146\/annurev.biochem.052308.114844","volume":"78","author":"ET Powers","year":"2009","unstructured":"Powers, E.T., Morimoto, R.I., Dillin, A., Kelly, J.W. & Balch, W.E. Biological and chemical approaches to diseases of proteostasis deficiency. Annu. Rev. Biochem. 78, 959\u2013991 (2009).","journal-title":"Annu. Rev. Biochem."},{"key":"BFnchembio275_CR6","doi-asserted-by":"publisher","first-page":"2639","DOI":"10.1016\/j.febslet.2009.07.014","volume":"583","author":"DM Hutt","year":"2009","unstructured":"Hutt, D.M., Powers, E.T. & Balch, W.E. The proteostasis boundary in misfolding diseases of membrane traffic. FEBS Lett. 583, 2639\u20132646 (2009).","journal-title":"FEBS Lett."},{"key":"BFnchembio275_CR7","doi-asserted-by":"publisher","first-page":"456","DOI":"10.1007\/s10545-006-0301-4","volume":"29","author":"N Gregersen","year":"2006","unstructured":"Gregersen, N. Protein misfolding disorders: pathogenesis and intervention. J. Inherit. Metab. Dis. 29, 456\u2013470 (2006).","journal-title":"J. Inherit. Metab. Dis."},{"key":"BFnchembio275_CR8","doi-asserted-by":"publisher","first-page":"84","DOI":"10.1038\/nbt1272","volume":"25","author":"PA Marks","year":"2007","unstructured":"Marks, P.A. & Breslow, R. Dimethyl sulfoxide to vorinostat: development of this histone deacetylase inhibitor as an anticancer drug. Nat. Biotechnol. 25, 84\u201390 (2007).","journal-title":"Nat. Biotechnol."},{"key":"BFnchembio275_CR9","doi-asserted-by":"publisher","first-page":"32","DOI":"10.1038\/nrg2485","volume":"10","author":"M Haberland","year":"2009","unstructured":"Haberland, M., Montgomery, R.L. & Olson, E.N. The many roles of histone deacetylases in development and physiology: implications for disease and therapy. Nat. Rev. Genet. 10, 32\u201342 (2009).","journal-title":"Nat. Rev. Genet."},{"key":"BFnchembio275_CR10","doi-asserted-by":"publisher","first-page":"151","DOI":"10.1016\/j.molcel.2006.12.008","volume":"25","author":"BT Scroggins","year":"2007","unstructured":"Scroggins, B.T. et al. An acetylation site in the middle domain of Hsp90 regulates chaperone function. Mol. Cell 25, 151\u2013159 (2007).","journal-title":"Mol. Cell"},{"key":"BFnchembio275_CR11","doi-asserted-by":"publisher","first-page":"1137","DOI":"10.1126\/science.1128294","volume":"313","author":"U Ozcan","year":"2006","unstructured":"Ozcan, U. et al. Chemical chaperones reduce ER stress and restore glucose homeostasis in a mouse model of type 2 diabetes. Science 313, 1137\u20131140 (2006).","journal-title":"Science"},{"key":"BFnchembio275_CR12","doi-asserted-by":"publisher","first-page":"694","DOI":"10.1016\/j.coi.2007.07.016","volume":"19","author":"IM Adcock","year":"2007","unstructured":"Adcock, I.M., Tsaprouni, L., Bhavsar, P. & Ito, K. Epigenetic regulation of airway inflammation. Curr. Opin. Immunol. 19, 694\u2013700 (2007).","journal-title":"Curr. Opin. Immunol."},{"key":"BFnchembio275_CR13","doi-asserted-by":"publisher","first-page":"862","DOI":"10.1038\/sj.bjp.0707165","volume":"150","author":"HS Lin","year":"2007","unstructured":"Lin, H.S. et al. Anti-rheumatic activities of histone deacetylase (HDAC) inhibitors in vivo in collagen-induced arthritis in rodents. Br. J. Pharmacol. 150, 862\u2013872 (2007).","journal-title":"Br. J. Pharmacol."},{"key":"BFnchembio275_CR14","doi-asserted-by":"publisher","first-page":"1063","DOI":"10.1126\/science.1165946","volume":"323","author":"SD Westerheide","year":"2009","unstructured":"Westerheide, S.D., Anckar, J., Stevens, S.M. Jr., Sistonen, L. & Morimoto, R.I. Stress-inducible regulation of heat shock factor 1 by the deacetylase SIRT1. Science 323, 1063\u20131066 (2009).","journal-title":"Science"},{"key":"BFnchembio275_CR15","doi-asserted-by":"publisher","first-page":"35826","DOI":"10.1074\/jbc.M104935200","volume":"276","author":"W Fischle","year":"2001","unstructured":"Fischle, W. et al. Human HDAC7 histone deacetylase activity is associated with HDAC3 in vivo. J. Biol. Chem. 276, 35826\u201335835 (2001).","journal-title":"J. Biol. Chem."},{"key":"BFnchembio275_CR16","doi-asserted-by":"publisher","first-page":"803","DOI":"10.1016\/j.cell.2006.09.043","volume":"127","author":"X Wang","year":"2006","unstructured":"Wang, X. et al. Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell 127, 803\u2013815 (2006).","journal-title":"Cell"},{"key":"BFnchembio275_CR17","doi-asserted-by":"publisher","first-page":"601","DOI":"10.1016\/j.molcel.2005.04.021","volume":"18","author":"JJ Kovacs","year":"2005","unstructured":"Kovacs, J.J. et al. HDAC6 regulates Hsp90 acetylation and chaperone-dependent activation of glucocorticoid receptor. Mol. Cell 18, 601\u2013607 (2005).","journal-title":"Mol. Cell"},{"key":"BFnchembio275_CR18","doi-asserted-by":"publisher","first-page":"601","DOI":"10.1113\/jphysiol.2005.096669","volume":"569","author":"Z Bebok","year":"2005","unstructured":"Bebok, Z. et al. Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers. J. Physiol. (Lond.) 569, 601\u2013615 (2005).","journal-title":"J. Physiol. (Lond.)"},{"key":"BFnchembio275_CR19","doi-asserted-by":"publisher","first-page":"6076","DOI":"10.1002\/j.1460-2075.1994.tb06954.x","volume":"13","author":"GL Lukacs","year":"1994","unstructured":"Lukacs, G.L. et al. Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP. EMBO J. 13, 6076\u20136086 (1994).","journal-title":"EMBO J."},{"key":"BFnchembio275_CR20","doi-asserted-by":"publisher","first-page":"2684","DOI":"10.1091\/mbc.e04-03-0176","volume":"15","author":"M Gentzsch","year":"2004","unstructured":"Gentzsch, M. et al. Endocytic trafficking routes of wild type and DeltaF508 cystic fibrosis transmembrane conductance regulator. Mol. Biol. Cell 15, 2684\u20132696 (2004).","journal-title":"Mol. Biol. Cell"},{"key":"BFnchembio275_CR21","doi-asserted-by":"publisher","first-page":"17335","DOI":"10.1073\/pnas.0706487104","volume":"104","author":"A Lahm","year":"2007","unstructured":"Lahm, A. et al. Unraveling the hidden catalytic activity of vertebrate class IIa histone deacetylases. Proc. Natl. Acad. Sci. USA 104, 17335\u201317340 (2007).","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"BFnchembio275_CR22","doi-asserted-by":"publisher","first-page":"1497","DOI":"10.1517\/13543784.14.12.1497","volume":"14","author":"PA Marks","year":"2005","unstructured":"Marks, P.A. & Dokmanovic, M. Histone deacetylase inhibitors: discovery and development as anticancer agents. Expert Opin. Investig. Drugs 14, 1497\u20131511 (2005).","journal-title":"Expert Opin. Investig. Drugs"},{"key":"BFnchembio275_CR23","doi-asserted-by":"publisher","first-page":"417","DOI":"10.1146\/annurev.biochem.73.011303.073651","volume":"73","author":"G Blander","year":"2004","unstructured":"Blander, G. & Guarente, L. The Sir2 family of protein deacetylases. Annu. Rev. Biochem. 73, 417\u2013435 (2004).","journal-title":"Annu. Rev. Biochem."},{"key":"BFnchembio275_CR24","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1016\/j.cbpa.2008.01.019","volume":"12","author":"JC Milne","year":"2008","unstructured":"Milne, J.C. & Denu, J.M. The Sirtuin family: therapeutic targets to treat diseases of aging. Curr. Opin. Chem. Biol. 12, 11\u201317 (2008).","journal-title":"Curr. Opin. Chem. Biol."},{"key":"BFnchembio275_CR25","doi-asserted-by":"publisher","first-page":"135","DOI":"10.1042\/BJ20080029","volume":"413","author":"E Caci","year":"2008","unstructured":"Caci, E. et al. Evidence for direct CFTR inhibition by CFTR(inh)-172 based on Arg347 mutagenesis. Biochem. J. 413, 135\u2013142 (2008).","journal-title":"Biochem. J."},{"key":"BFnchembio275_CR26","first-page":"319","volume":"356","author":"S Ekins","year":"2007","unstructured":"Ekins, S., Nikolsky, Y., Bugrim, A., Kirillov, E. & Nikolskaya, T. Pathway mapping tools for analysis of high content data. Methods Mol. Biol. 356, 319\u2013350 (2007).","journal-title":"Methods Mol. Biol."},{"key":"BFnchembio275_CR27","doi-asserted-by":"publisher","first-page":"36856","DOI":"10.1074\/jbc.M607085200","volume":"281","author":"F Sun","year":"2006","unstructured":"Sun, F. et al. Derlin-1 promotes the efficient degradation of cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants. J. Biol. Chem. 281, 36856\u201336863 (2006).","journal-title":"J. Biol. Chem."},{"key":"BFnchembio275_CR28","doi-asserted-by":"publisher","first-page":"1878","DOI":"10.1111\/j.1600-0854.2008.00806.x","volume":"9","author":"X Wang","year":"2008","unstructured":"Wang, X., Koulov, A.V., Kellner, W.A., Riordan, J.R. & Balch, W.E. Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking. Traffic 9, 1878\u20131893 (2008).","journal-title":"Traffic"},{"key":"BFnchembio275_CR29","doi-asserted-by":"publisher","first-page":"65","DOI":"10.1083\/jcb.200401035","volume":"167","author":"X Wang","year":"2004","unstructured":"Wang, X. et al. COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code. J. Cell Biol. 167, 65\u201374 (2004).","journal-title":"J. Cell Biol."},{"key":"BFnchembio275_CR30","doi-asserted-by":"publisher","first-page":"809","DOI":"10.1016\/j.cell.2007.10.025","volume":"131","author":"RL Wiseman","year":"2007","unstructured":"Wiseman, R.L., Powers, E.T., Buxbaum, J.N., Kelly, J.W. & Balch, W.E. An adaptable standard for protein export from the endoplasmic reticulum. Cell 131, 809\u2013821 (2007).","journal-title":"Cell"},{"key":"BFnchembio275_CR31","doi-asserted-by":"publisher","first-page":"426","DOI":"10.1038\/nrm1949","volume":"7","author":"WB Guggino","year":"2006","unstructured":"Guggino, W.B. & Stanton, B.A. New insights into cystic fibrosis: molecular switches that regulate CFTR. Nat. Rev. Mol. Cell Biol. 7, 426\u2013436 (2006).","journal-title":"Nat. Rev. Mol. Cell Biol."},{"key":"BFnchembio275_CR32","doi-asserted-by":"publisher","first-page":"2525","DOI":"10.1158\/1535-7163.MCT-07-0251","volume":"6","author":"M Dokmanovic","year":"2007","unstructured":"Dokmanovic, M. et al. Histone deacetylase inhibitors selectively suppress expression of HDAC7. Mol. Cancer Ther. 6, 2525\u20132534 (2007).","journal-title":"Mol. Cancer Ther."},{"key":"BFnchembio275_CR33","doi-asserted-by":"publisher","first-page":"321","DOI":"10.1016\/j.cell.2006.05.040","volume":"126","author":"S Chang","year":"2006","unstructured":"Chang, S. et al. Histone deacetylase 7 maintains vascular integrity by repressing matrix metalloproteinase 10. Cell 126, 321\u2013334 (2006).","journal-title":"Cell"},{"key":"BFnchembio275_CR34","doi-asserted-by":"publisher","first-page":"5184","DOI":"10.1128\/MCB.02091-06","volume":"27","author":"HG Kasler","year":"2007","unstructured":"Kasler, H.G. & Verdin, E. Histone deacetylase 7 functions as a key regulator of genes involved in both positive and negative selection of thymocytes. Mol. Cell. Biol. 27, 5184\u20135200 (2007).","journal-title":"Mol. Cell. Biol."},{"key":"BFnchembio275_CR35","doi-asserted-by":"publisher","first-page":"479","DOI":"10.1002\/ppul.20168","volume":"39","author":"MD Amaral","year":"2005","unstructured":"Amaral, M.D. Processing of CFTR: traversing the cellular maze\u2013how much CFTR needs to go through to avoid cystic fibrosis? Pediatr. Pulmonol. 39, 479\u2013491 (2005).","journal-title":"Pediatr. Pulmonol."},{"key":"BFnchembio275_CR36","doi-asserted-by":"publisher","first-page":"11401","DOI":"10.1074\/jbc.M110263200","volume":"277","author":"JS Yoo","year":"2002","unstructured":"Yoo, J.S. et al. Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator through the early secretory pathway. J. Biol. Chem. 277, 11401\u201311409 (2002).","journal-title":"J. Biol. Chem."},{"key":"BFnchembio275_CR37","doi-asserted-by":"publisher","first-page":"835","DOI":"10.1517\/14728222.11.6.835","volume":"11","author":"A Mai","year":"2007","unstructured":"Mai, A. The therapeutic uses of chromatin-modifying agents. Expert Opin. Ther. Targets 11, 835\u2013851 (2007).","journal-title":"Expert Opin. Ther. Targets"},{"key":"BFnchembio275_CR38","doi-asserted-by":"publisher","first-page":"551","DOI":"10.1038\/nchembio815","volume":"2","author":"D Herman","year":"2006","unstructured":"Herman, D. et al. Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia. Nat. Chem. Biol. 2, 551\u2013558 (2006).","journal-title":"Nat. Chem. Biol."},{"key":"BFnchembio275_CR39","doi-asserted-by":"publisher","first-page":"1111","DOI":"10.1124\/mol.107.036772","volume":"72","author":"A Mai","year":"2007","unstructured":"Mai, A. et al. Identification of two new synthetic histone deacetylase inhibitors that modulate globin gene expression in erythroid cells from healthy donors and patients with thalassemia. Mol. Pharmacol. 72, 1111\u20131123 (2007).","journal-title":"Mol. Pharmacol."},{"key":"BFnchembio275_CR40","doi-asserted-by":"publisher","first-page":"555","DOI":"10.1016\/j.tibs.2007.09.008","volume":"32","author":"CH Westphal","year":"2007","unstructured":"Westphal, C.H., Dipp, M.A. & Guarente, L. A therapeutic role for sirtuins in diseases of aging? Trends Biochem. Sci. 32, 555\u2013560 (2007).","journal-title":"Trends Biochem. Sci."},{"key":"BFnchembio275_CR41","doi-asserted-by":"publisher","first-page":"2830","DOI":"10.1523\/JNEUROSCI.3344-05.2006","volume":"26","author":"EA Bates","year":"2006","unstructured":"Bates, E.A., Victor, M., Jones, A.K., Shi, Y. & Hart, A.C. Differential contributions of Caenorhabditis elegans histone deacetylases to huntingtin polyglutamine toxicity. J. Neurosci. 26, 2830\u20132838 (2006).","journal-title":"J. Neurosci."},{"key":"BFnchembio275_CR42","doi-asserted-by":"publisher","first-page":"2457","DOI":"10.1172\/JCI119788","volume":"100","author":"RC Rubenstein","year":"1997","unstructured":"Rubenstein, R.C., Egan, M.E. & Zeitlin, P.L. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR. J. Clin. Invest. 100, 2457\u20132465 (1997).","journal-title":"J. Clin. Invest."},{"key":"BFnchembio275_CR43","doi-asserted-by":"publisher","first-page":"562","DOI":"10.1021\/pr050319o","volume":"5","author":"OV Singh","year":"2006","unstructured":"Singh, O.V. et al. Pharmacoproteomics of 4-phenylbutyrate-treated IB3\u20131 cystic fibrosis bronchial epithelial cells. J. Proteome Res. 5, 562\u2013571 (2006).","journal-title":"J. Proteome Res."},{"key":"BFnchembio275_CR44","doi-asserted-by":"publisher","first-page":"484","DOI":"10.1164\/ajrccm.157.2.9706088","volume":"157","author":"RC Rubenstein","year":"1998","unstructured":"Rubenstein, R.C. & Zeitlin, P.L. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: partial restoration of nasal epithelial CFTR function. Am. J. Respir. Crit. Care Med. 157, 484\u2013490 (1998).","journal-title":"Am. J. Respir. Crit. Care Med."},{"issue":"suppl. 5","key":"BFnchembio275_CR45","first-page":"453","volume":"58","author":"RM Mroz","year":"2007","unstructured":"Mroz, R.M., Noparlik, J., Chyczewska, E., Braszko, J.J. & Holownia, A. Molecular basis of chronic inflammation in lung diseases: new therapeutic approach. J. Physiol. Pharmacol. 58 (suppl. 5): 453\u2013460 (2007).","journal-title":"J. Physiol. Pharmacol."},{"key":"BFnchembio275_CR46","first-page":"427","volume":"3","author":"DC Lei","year":"1996","unstructured":"Lei, D.C. et al. Episomal expression of wild-type CFTR corrects cAMP-dependent chloride transport in respiratory epithelial cells. Gene Ther. 3, 427\u2013436 (1996).","journal-title":"Gene Ther."},{"key":"BFnchembio275_CR47","doi-asserted-by":"publisher","first-page":"C461","DOI":"10.1152\/ajpcell.2000.279.2.C461","volume":"279","author":"DC Devor","year":"2000","unstructured":"Devor, D.C., Bridges, R.J. & Pilewski, J.M. Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia. Am. J. Physiol. Cell Physiol. 279, C461\u2013C479 (2000).","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"BFnchembio275_CR48","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1085\/jgp.200409124","volume":"125","author":"MB Butterworth","year":"2005","unstructured":"Butterworth, M.B., Edinger, R.S., Johnson, J.P. & Frizzell, R.A. Acute ENaC stimulation by cAMP in a kidney cell line is mediated by exocytic insertion from a recycling channel pool. J. Gen. Physiol. 125, 81\u2013101 (2005).","journal-title":"J. Gen. Physiol."},{"key":"BFnchembio275_CR49","doi-asserted-by":"publisher","first-page":"27942","DOI":"10.1074\/jbc.M606449200","volume":"281","author":"MM Myerburg","year":"2006","unstructured":"Myerburg, M.M. et al. Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: a mechanism for sodium hyperabsorption in cystic fibrosis. J. Biol. Chem. 281, 27942\u201327949 (2006).","journal-title":"J. Biol. Chem."},{"key":"BFnchembio275_CR50","doi-asserted-by":"publisher","first-page":"L932","DOI":"10.1152\/ajplung.00437.2007","volume":"294","author":"MM Myerburg","year":"2008","unstructured":"Myerburg, M.M. et al. Prostasin expression is regulated by airway surface liquid volume and is increased in cystic fibrosis. Am. J. Physiol. Lung Cell. Mol. Physiol. 294, L932\u2013L941 (2008).","journal-title":"Am. J. Physiol. Lung Cell. Mol. Physiol."}],"container-title":["Nature Chemical Biology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/www.nature.com\/articles\/nchembio.275.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nchembio.275","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/www.nature.com\/articles\/nchembio.275.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,5,18]],"date-time":"2023-05-18T19:47:22Z","timestamp":1684439242000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/nchembio.275"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,12,6]]},"references-count":50,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2010,1]]}},"alternative-id":["BFnchembio275"],"URL":"https:\/\/doi.org\/10.1038\/nchembio.275","relation":{},"ISSN":["1552-4450","1552-4469"],"issn-type":[{"value":"1552-4450","type":"print"},{"value":"1552-4469","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,12,6]]}}}