Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer
dc.contributor.author | Drumm, Mitchell L. | en_US |
dc.contributor.author | Pope, Heidi A. | en_US |
dc.contributor.author | Cliff, William H. | en_US |
dc.contributor.author | Rommens, Johanna M. | en_US |
dc.contributor.author | Marvin, Sheila A. | en_US |
dc.contributor.author | Tsui, Lap-Chee | en_US |
dc.contributor.author | Collins, Francis S. | en_US |
dc.contributor.author | Frizzell, Raymond A. | en_US |
dc.contributor.author | Wilson, James M. | en_US |
dc.date.accessioned | 2006-04-10T13:36:34Z | |
dc.date.available | 2006-04-10T13:36:34Z | |
dc.date.issued | 1990-09-21 | en_US |
dc.identifier.citation | Drumm, 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.uri | http://www.sciencedirect.com/science/article/B6WSN-4C5PJSG-8B/2/1b77059487c3e19c743ea84d71816f03 | en_US |
dc.identifier.uri | https://hdl.handle.net/2027.42/28383 | |
dc.identifier.uri | http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=1698126&dopt=citation | en_US |
dc.description.abstract | We 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.extent | 1107903 bytes | |
dc.format.extent | 3118 bytes | |
dc.format.mimetype | application/pdf | |
dc.format.mimetype | text/plain | |
dc.language.iso | en_US | |
dc.publisher | Elsevier | en_US |
dc.title | Correction of the cystic fibrosis defect in vitro by retrovirus-mediated gene transfer | en_US |
dc.type | Article | en_US |
dc.rights.robots | IndexNoFollow | en_US |
dc.subject.hlbsecondlevel | Molecular, Cellular and Developmental Biology | en_US |
dc.subject.hlbtoplevel | Science | en_US |
dc.subject.hlbtoplevel | Health Sciences | en_US |
dc.description.peerreviewed | Peer Reviewed | en_US |
dc.contributor.affiliationum | Department 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, USA | en_US |
dc.contributor.affiliationum | Department 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, USA | en_US |
dc.contributor.affiliationum | Department 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, USA | en_US |
dc.contributor.affiliationum | Department 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, USA | en_US |
dc.contributor.affiliationother | Gregory Fleming James Cystic Fibrosis Research Center and Department of Physiology and Biophysics University of Alabama at Birmingham UAB Station, Birmingham, Alabama 35294, USA | en_US |
dc.contributor.affiliationother | Department of Genetics Research Institute The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada | en_US |
dc.contributor.affiliationother | Gregory Fleming James Cystic Fibrosis Research Center and Department of Physiology and Biophysics University of Alabama at Birmingham UAB Station, Birmingham, Alabama 35294, USA | en_US |
dc.contributor.affiliationother | Department of Genetics Research Institute The Hospital for Sick Children, Toronto, Ontario M5G 1X8, Canada | en_US |
dc.contributor.affiliationother | Gregory Fleming James Cystic Fibrosis Research Center and Department of Physiology and Biophysics University of Alabama at Birmingham UAB Station, Birmingham, Alabama 35294, USA | en_US |
dc.identifier.pmid | 1698126 | en_US |
dc.description.bitstreamurl | http://deepblue.lib.umich.edu/bitstream/2027.42/28383/1/0000156.pdf | en_US |
dc.identifier.doi | http://dx.doi.org/10.1016/0092-8674(90)90398-X | en_US |
dc.identifier.source | Cell | en_US |
dc.owningcollname | Interdisciplinary and Peer-Reviewed |
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