{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T13:45:14Z","timestamp":1790862314111,"version":"4.1.0"},"reference-count":64,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2007,7,24]],"date-time":"2007-07-24T00:00:00Z","timestamp":1185235200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Journal Cellular Physiology"],"published-print":{"date-parts":[[2008,2]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Cystic fibrosis (CF) is a fatal inherited disease caused by the absence or dysfunction of the CF transmembrane conductance regulator (CFTR) Cl<jats:sup>\u2212<\/jats:sup> channel. About 70% of CF patients are exocrine pancreatic insufficient due to failure of the pancreatic ducts to secrete a HCO<jats:inline-graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"graphic\/tex2gif-stack-1.gif\" xlink:title=\"urn:x-wiley:00219541:media:JCP21220:tex2gif-stack-1\"\/>\u2010rich fluid. Our aim in this study was to investigate the potential of a recombinant Sendai virus (SeV) vector to introduce normal CFTR into human CF pancreatic duct (CFPAC\u20101) cells, and to assess the effect of CFTR gene transfer on the key transporters involved in HCO<jats:inline-graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"graphic\/tex2gif-stack-2.gif\" xlink:title=\"urn:x-wiley:00219541:media:JCP21220:tex2gif-stack-2\"\/> transport. Using polarized cultures of homozygous F508del CFPAC\u20101 cells as a model for the human CF pancreatic ductal epithelium we showed that SeV was an efficient gene transfer agent when applied to the apical membrane. The presence of functional CFTR was confirmed using iodide efflux assay. CFTR expression had no effect on cell growth, monolayer integrity, and mRNA levels for key transporters in the duct cell (pNBC, AE2, NHE2, NHE3, DRA, and PAT\u20101), but did upregulate the activity of apical Cl<jats:sup>\u2212<\/jats:sup>\/HCO<jats:inline-graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"graphic\/tex2gif-stack-3.gif\" xlink:title=\"urn:x-wiley:00219541:media:JCP21220:tex2gif-stack-3\"\/> and Na<jats:sup>+<\/jats:sup>\/H<jats:sup>+<\/jats:sup> exchangers (NHEs). In CFTR\u2010corrected cells, apical Cl<jats:sup>\u2212<\/jats:sup>\/HCO<jats:inline-graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"graphic\/tex2gif-stack-4.gif\" xlink:title=\"urn:x-wiley:00219541:media:JCP21220:tex2gif-stack-4\"\/> exchange activity was further enhanced by cAMP, a key feature exhibited by normal pancreatic duct cells. The cAMP stimulated Cl<jats:sup>\u2212<\/jats:sup>\/HCO<jats:inline-graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"graphic\/tex2gif-stack-5.gif\" xlink:title=\"urn:x-wiley:00219541:media:JCP21220:tex2gif-stack-5\"\/> exchange was inhibited by dihydro\u20104,4\u2032\u2010diisothiocyanostilbene\u20102,2\u2032\u2010disulfonic acid (H<jats:sub>2<\/jats:sub>\u2010DIDS), but not by a specific CFTR inhibitor, CFTR<jats:sub>inh<\/jats:sub>\u2010172. Our data show that SeV vector is a potential CFTR gene transfer agent for human pancreatic duct cells and that expression of CFTR in CF cells is associated with a restoration of Cl<jats:sup>\u2212<\/jats:sup> and HCO<jats:inline-graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"graphic\/tex2gif-stack-6.gif\" xlink:title=\"urn:x-wiley:00219541:media:JCP21220:tex2gif-stack-6\"\/> transport at the apical membrane. J. Cell. Physiol. 214: 442\u2013455, 2008. \u00a9 2007 Wiley\u2010Liss, Inc.<\/jats:p>","DOI":"10.1002\/jcp.21220","type":"journal-article","created":{"date-parts":[[2007,7,24]],"date-time":"2007-07-24T16:48:20Z","timestamp":1185295700000},"page":"442-455","source":"Crossref","is-referenced-by-count":31,"title":["CFTR gene transfer to human cystic fibrosis pancreatic duct cells using a Sendai virus 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