阿米洛利
上皮钠通道
囊性纤维化跨膜传导调节器
调节器
囊性纤维化
钠
环磷酸腺苷
钠通道
氯离子通道
内科学
化学
细胞生物学
内分泌学
离子通道
腺苷
生物
生物化学
基因
医学
受体
有机化学
作者
M. Jackson Stutts,Cecilia M. Canessa,JC Olsen,Melissa F Hamrick,J. A. Cohn,B. C. Rossier,R. C. Boucher
出处
期刊:Science
[American Association for the Advancement of Science (AAAS)]
日期:1995-08-11
卷期号:269 (5225): 847-850
被引量:1033
标识
DOI:10.1126/science.7543698
摘要
Cystic fibrosis transmembrane regulator (CFTR), the gene product that is mutated in cystic fibrosis (CF) patients, has a well-recognized function as a cyclic adenosine 3',5'-monophosphate (cAMP)-regulated chloride channel, but this property does not account for the abnormally high basal rate and cAMP sensitivity of sodium ion absorption in CF airway epithelia. Expression of complementary DNAs for rat epithelial Na+ channel (rENaC) alone in Madin Darby canine kidney (MDCK) epithelial cells generated large amiloride-sensitive sodium currents that were stimulated by cAMP, whereas coexpression of human CFTR with rENaC generated smaller basal sodium currents that were inhibited by cAMP. Parallel studies that measured regulation of sodium permeability in fibroblasts showed similar results. In CF airway epithelia, the absence of this second function of CFTR as a cAMP-dependent regulator likely accounts for abnormal sodium transport.
科研通智能强力驱动
Strongly Powered by AbleSci AI