Defective Epithelial Chloride Transport in a Gene-Targeted Mouse Model of Cystic Fibrosis
作者： L. L. Clarke , B. R. Grubb , S. E. Gabriel , O. Smithies , B. H. Koller
DOI: 10.1126/SCIENCE.257.5073.1125
关键词:
摘要: The cystic fibrosis transmembrane conductance regulator (CFTR) gene encodes an adenosine 39, 59-monophosphate (cyclic AMP)—activated chloride channel. In (CF) patients, loss of CFTR function because a genetic mutation results in defective cyclic AMP—mediated secretion across epithelia. Because their potential role as animal model for CF, mice with targeted disruption the murine [CFTR(-/-)] were tested abnormalities epithelial transport. both freshly excised tissue from intestine and cultured epithelia proximal airways, AMP—activated secretory response was absent CFTR(-/-) compared to littermate controls. Thus, transport predicted studies human CF
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