{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T09:01:36Z","timestamp":1778662896856,"version":"3.51.4"},"reference-count":34,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2011,10,20]],"date-time":"2011-10-20T00:00:00Z","timestamp":1319068800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Experimental Physiology"],"published-print":{"date-parts":[[2012,1]]},"abstract":"<jats:p>The cystic fibrosis transmembrane conductance regulator (CFTR) is both an anion channel and a regulator of other transport proteins. Mutations in the\u2002<jats:italic>CFTR<\/jats:italic>\u2002gene underlie the human disease, cystic fibrosis. The most common\u2002<jats:italic>CFTR<\/jats:italic>\u2002mutation, \u0394F508, produces a misfolded protein which traffics improperly. The availability of transgenic\u2002<jats:italic>CFTR<\/jats:italic><jats:sup>\u0394F508\/\u0394F508<\/jats:sup> pigs allows measurement of the impact of \u0394F508 in native tissue. Thyroid epithelia respond to cAMP\u2010elevating agents by increasing anion transport, a process reliant on functional CFTR. To assess whether endogenous levels of \u0394F508\u2010CFTR mediate thyroid transport, primary thyroid epithelial cultures (pThECs) were grown from newborn\u2002<jats:italic>CFTR<\/jats:italic><jats:sup>+\/+<\/jats:sup> (wild\u2010type) and\u2002<jats:italic>CFTR<\/jats:italic><jats:sup>\u0394F508\/\u0394F508<\/jats:sup> (\u0394F) pig thyroids and the stimulated, secretory components of short\u2010circuit current (<jats:italic>I<\/jats:italic><jats:sub>sc<\/jats:sub>) compared. Surface biotinylation studies assessed the surface presentation of \u0394F508\u2010CFTR. Baseline\u2002<jats:italic>I<\/jats:italic><jats:sub>sc<\/jats:sub> levels of both wild\u2010type and \u0394F pThECs consisted of an amiloride\u2010sensitive component. In \u0394F pThECs, this mirrored previous measurements in\u2002<jats:italic>CFTR<\/jats:italic><jats:sup>\u2212\/\u2212<\/jats:sup> (knockout) pThECs. Surprisingly, elevation of cAMP transiently increased\u2002<jats:italic>I<\/jats:italic><jats:sub>sc<\/jats:sub> to peak levels \u223c65% of those achieved by wild\u2010type. In contrast, knockout pThECs were indifferent to cAMP activation. In \u0394F pThECs, total \u0394F508\u2010CFTR expression was \u223c9% that of wild\u2010type, consistent with misfolding and enhanced degradation. Surface biotinylation studies indicated that \u223c4% of the total \u0394F508 resided at the surface and did not increase with cAMP elevation. The present findings show that low endogenous levels of pig \u0394F508\u2010CFTR can mediate substantial anion transport by thyroid epithelia. These data suggest that both wild\u2010type and \u0394F508\u2010CFTR regulate additional thyroid transporters, and together co\u2010ordinate the overall\u2002<jats:italic>I<\/jats:italic><jats:sub>sc<\/jats:sub> response.<\/jats:p>","DOI":"10.1113\/expphysiol.2011.060756","type":"journal-article","created":{"date-parts":[[2011,9,24]],"date-time":"2011-09-24T08:05:59Z","timestamp":1316851559000},"page":"115-124","source":"Crossref","is-referenced-by-count":5,"title":["Endogenous surface expression of \u0394F508\u2010CFTR mediates cAMP\u2010stimulated Cl<sup>\u2212<\/sup> current in\u2002<i>CFTR<\/i><sup>\u0394F508\/\u0394F508<\/sup> pig thyroid epithelial cells"],"prefix":"10.1113","volume":"97","author":[{"given":"Yonghai","family":"Li","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Suhasini","family":"Ganta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peying","family":"Fong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2011,10,20]]},"reference":[{"key":"e_1_2_5_2_1","first-page":"114","article-title":"Increased serum TSH response to TRH in cystic fibrosis","volume":"10","author":"Baran D","year":"1979","journal-title":"Monogr Paediatr"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cell.2010.11.029"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(90)90148-8"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jmb.2006.10.086"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1038\/354526a0"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00442510"},{"key":"e_1_2_5_8_1","doi-asserted-by":"publisher","DOI":"10.1038\/358761a0"},{"key":"e_1_2_5_9_1","doi-asserted-by":"crossref","first-page":"C1299","DOI":"10.1152\/ajpcell.1997.272.4.C1299","article-title":"Expression of CFTR in human and bovine thyroid epithelium","volume":"272","author":"Devuyst O","year":"1997","journal-title":"Am J Physiol Cell Physiol"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-5193(05)80362-5"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncb0404-292"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00424-002-0956-0"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ijc.24679"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncb1115"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1113\/jphysiol.2009.182246"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1113\/expphysiol.2010.054700"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbrc.2008.06.009"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.1998.275.4.C913"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1042\/BJ20081869"},{"key":"e_1_2_5_20_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.3001868"},{"key":"e_1_2_5_21_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0706974104"},{"key":"e_1_2_5_22_1","doi-asserted-by":"publisher","DOI":"10.1054\/mehy.1999.0806"},{"key":"e_1_2_5_23_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajpcell.00404.2009"},{"key":"e_1_2_5_24_1","doi-asserted-by":"publisher","DOI":"10.1152\/ajplung.90203.2008"},{"key":"e_1_2_5_25_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI34773"},{"key":"e_1_2_5_26_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.1163600"},{"key":"e_1_2_5_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pupt.2010.02.001"},{"key":"e_1_2_5_28_1","doi-asserted-by":"publisher","DOI":"10.1002\/0470029579.ch12"},{"key":"e_1_2_5_29_1","doi-asserted-by":"crossref","first-page":"L405","DOI":"10.1152\/ajplung.1994.266.4.L405","article-title":"Expression of cystic fibrosis transmembrane conductance regulator in a model epithelium","volume":"266","author":"Sheppard DN","year":"1994","journal-title":"Am J Physiol Lung Cell Mol Physiol"},{"key":"e_1_2_5_30_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2222.1998.00312.x"},{"key":"e_1_2_5_31_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.3000928"},{"key":"e_1_2_5_32_1","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.110.4.341"},{"key":"e_1_2_5_33_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2010.08.001"},{"key":"e_1_2_5_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1569-1993(11)60020-9"},{"key":"e_1_2_5_35_1","first-page":"163","article-title":"Human thyroid epithelial cells","volume":"75","author":"Williams DW","year":"1997","journal-title":"Methods Mol Biol"}],"container-title":["Experimental Physiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1113%2Fexpphysiol.2011.060756","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1113%2Fexpphysiol.2011.060756","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/physoc.onlinelibrary.wiley.com\/doi\/pdf\/10.1113\/expphysiol.2011.060756","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,30]],"date-time":"2023-10-30T06:11:51Z","timestamp":1698646311000},"score":1,"resource":{"primary":{"URL":"https:\/\/physoc.onlinelibrary.wiley.com\/doi\/10.1113\/expphysiol.2011.060756"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,10,20]]},"references-count":34,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2012,1]]}},"alternative-id":["10.1113\/expphysiol.2011.060756"],"URL":"https:\/\/doi.org\/10.1113\/expphysiol.2011.060756","archive":["Portico"],"relation":{},"ISSN":["0958-0670","1469-445X"],"issn-type":[{"value":"0958-0670","type":"print"},{"value":"1469-445X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2011,10,20]]}}}