Article
Mechanosensitivity of wild-type and G551D cystic fibrosis transmembrane conductance regulator (CFTR) controls regulatory volume decrease in simple epithelia.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Apr 2016
Xie Changyan, Cao Xu, Chen Xibing, Wang Dong, Zhang Wei Kevin, Sun Ying, Hu Wenbao, Zhou Zijing, Wang Yan, Huang Pingbo
Abstract excerpt
Mutations of cystic fibrosis transmembrane conductance regulator (CFTR), an epithelial ligand-gated anion channel, are associated with the lethal genetic disease cystic fibrosis. The CFTR G551D mutation impairs ATP hydrolysis and thereby makes CFTR refractory to cAMP stimulation. Both wild-type (WT) and G551D CFTR have been implicated in regulatory volume decrease (RVD), but the underlying mechanism remains...
Topics
- Animals
- CHO Cells
- Cell Line, Tumor
- Cell Size
- Cricetinae
- Cricetulus
- Cystic Fibrosis Transmembrane Conductance Regulator
- Epithelial Cells
- Humans
- Hypotonic Solutions
- Ion Channel Gating
- Mechanoreceptors