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Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer

dc.contributor.authorDrumm, Mitchell L.en_US
dc.contributor.authorPope, Heidi A.en_US
dc.contributor.authorCliff, William H.en_US
dc.contributor.authorRommens, Johanna M.en_US
dc.contributor.authorMarvin, Sheila A.en_US
dc.contributor.authorTsui, Lap-Cheeen_US
dc.contributor.authorCollins, Francis S.en_US
dc.contributor.authorFrizzell, Raymond A.en_US
dc.contributor.authorWilson, James M.en_US
dc.date.accessioned2006-04-10T13:36:34Z
dc.date.available2006-04-10T13:36:34Z
dc.date.issued1990-09-21en_US
dc.identifier.citationDrumm, Mitchell L., Pope, Heidi A., Cliff, William H., Rommens, Johanna M., Marvin, Sheila A., Tsui, Lap-Chee, Collins, Francis S., Frizzell, Raymond A., Wilson, James M. (1990/09/21)."Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer." Cell 62(6): 1227-1233. <http://hdl.handle.net/2027.42/28383>en_US
dc.identifier.urihttp://www.sciencedirect.com/science/article/B6WSN-4C5PJSG-8B/2/1b77059487c3e19c743ea84d71816f03en_US
dc.identifier.urihttps://hdl.handle.net/2027.42/28383
dc.identifier.urihttp://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=1698126&dopt=citationen_US
dc.description.abstractWe have used retrovirus-mediated gene transfer to demonstrate complementation of the cystic fibrosis (CF) defect in vitro. Amphotropic retroviruses were used to transduce a functional cystic fibrosis transmembrane conductance regulator (CFTR) cDNA into CFPAC-1, a pancreatic adenocarcinoma cell line derived from a patient with CF that stably expresses the chloride transport abnormalities characteristic of CF. CFPAC-1 cells were exposed to control virus (PLJ) and CFTR-expressing virus (PLJ-CFTR); viral-transduced clones were isolated and subjected to molecular and physiologic analysis. RNA analysis detected a viral-derived CFTR transcript in all of the PLJ-CFTR clones that contained unrearranged proviral sequences. Agents that increase intracellular cAMP stimulated 125I efflux in PLJ-CFTR clones but not PLJ clones. Whole-cell patch-clamp performed on three responding clones showed that the anion efflux responses were due to cAMP stimulation of Cl conductance. Our findings indicate that expression of the normal CFTR gene confers cAMP-dependent Cl channel regulation on CF epithelial cells.en_US
dc.format.extent1107903 bytes
dc.format.extent3118 bytes
dc.format.mimetypeapplication/pdf
dc.format.mimetypetext/plain
dc.language.isoen_US
dc.publisherElsevieren_US
dc.titleCorrection of the cystic fibrosis defect in vitro by retrovirus-mediated gene transferen_US
dc.typeArticleen_US
dc.rights.robotsIndexNoFollowen_US
dc.subject.hlbsecondlevelMolecular, Cellular and Developmental Biologyen_US
dc.subject.hlbtoplevelScienceen_US
dc.subject.hlbtoplevelHealth Sciencesen_US
dc.description.peerreviewedPeer Revieweden_US
dc.contributor.affiliationumDepartment of Human Genetics Howard Hughes Medical Institute University of Michigan Medical School, Ann Arbor, Michigan 48109-0650, USA; Department of Human Genetics Howard Hughes Medical Institute University of Michigan Medical School, Ann Arbor, Michigan 48109-0650, USAen_US
dc.contributor.affiliationumDepartment of Biological Chemistry Howard Hughes Medical Institute University of Michigan Medical School, Ann Arbor, Michigan 48109-0650, USA; Department of Biological Chemistry Howard Hughes Medical Institute University of Michigan Medical School, Ann Arbor, Michigan 48109-0650, USAen_US
dc.contributor.affiliationumDepartment of Human Genetics Howard Hughes Medical Institute University of Michigan Medical School, Ann Arbor, Michigan 48109-0650, USA; Department of Human Genetics Howard Hughes Medical Institute University of Michigan Medical School, Ann Arbor, Michigan 48109-0650, USAen_US
dc.contributor.affiliationumDepartment of Biological Chemistry Howard Hughes Medical Institute University of Michigan Medical School, Ann Arbor, Michigan 48109-0650, USA; Department of Biological Chemistry Howard Hughes Medical Institute University of Michigan Medical School, Ann Arbor, Michigan 48109-0650, USAen_US
dc.contributor.affiliationotherGregory Fleming James Cystic Fibrosis Research Center and Department of Physiology and Biophysics University of Alabama at Birmingham UAB Station, Birmingham, Alabama 35294, USAen_US
dc.contributor.affiliationotherDepartment of Genetics Research Institute The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canadaen_US
dc.contributor.affiliationotherGregory Fleming James Cystic Fibrosis Research Center and Department of Physiology and Biophysics University of Alabama at Birmingham UAB Station, Birmingham, Alabama 35294, USAen_US
dc.contributor.affiliationotherDepartment of Genetics Research Institute The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canadaen_US
dc.contributor.affiliationotherGregory Fleming James Cystic Fibrosis Research Center and Department of Physiology and Biophysics University of Alabama at Birmingham UAB Station, Birmingham, Alabama 35294, USAen_US
dc.identifier.pmid1698126en_US
dc.description.bitstreamurlhttp://deepblue.lib.umich.edu/bitstream/2027.42/28383/1/0000156.pdfen_US
dc.identifier.doihttp://dx.doi.org/10.1016/0092-8674(90)90398-Xen_US
dc.identifier.sourceCellen_US
dc.owningcollnameInterdisciplinary and Peer-Reviewed


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