| | |  | | | | Record from Web of Science® | | | | | | |
| DEFECTIVE EPITHELIAL CHLORIDE TRANSPORT IN A GENE-TARGETED MOUSE MODEL OF CYSTIC-FIBROSIS |
|
|
| Author(s): CLARKE LL, GRUBB BR, GABRIEL SE, SMITHIES O, KOLLER BH, BOUCHER RC |
| Source: SCIENCE Volume: 257 Issue: 5073 Pages: 1125-1128 Published: AUG 21 1992 |
| Times Cited: 210 References: 36 |
| Abstract: The cystic fibrosis transmembrane conductance regulator (CFTR) gene encodes an adenosine 3',5'-monophosphate (cyclic AMP)-activated chloride channel. In cystic fibrosis (CF) patients, loss of CFTR function because of a genetic mutation results in defective cyclic AMP-mediated chloride secretion across epithelia. Because of their potential role as an animal model for CF, mice with targeted disruption of the murine CFTR gene [CFTR(-/-)] were tested for abnormalities in epithelial chloride transport. In both freshly excised tissue from the intestine and in cultured epithelia from the proximal airways, the cyclic AMP-activated chloride secretory response was absent in CFTR(-/-) mice as compared to littermate controls. Thus, disruption of the murine CFTR gene results in the chloride transport abnormalities predicted from studies of human CF epithelia. |
| Document Type: Article |
| Language: English |
Addresses:
1. UNIV N CAROLINA, DEPT MED, DIV PULM DIS, CHAPEL HILL, NC 27514 USA |
| Publisher: AMER ASSOC ADVANCEMENT SCIENCE, 1200 NEW YORK AVE, NW, WASHINGTON, DC 20005 |
| Subject Category: Multidisciplinary Sciences |
| IDS Number: JJ884 |
| ISSN: 0036-8075 |
|
| | | | | | | | | Record from Web of Science® | | | | | | | | | |