| First Author | Clarke LL | Year | 1992 |
| Journal | Science | Volume | 257 |
| Issue | 5073 | Pages | 1125-8 |
| PubMed ID | 1380724 | Mgi Jnum | J:23817 |
| Mgi Id | MGI:71691 | Doi | 10.1126/science.257.5073.1125 |
| Citation | Clarke LL, et al. (1992) Defective epithelial chloride transport in a gene-targeted mouse model of cystic fibrosis. Science 257(5073):1125-8 |
| abstractText | 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. |
