Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) chloride channel plays a critical role in regulating transepithelial movement of water and electrolyte in exocrine tissues. Malfunction of the channel because of mutations of the cftr gene results in CF, the most prevalent lethal genetic disease among Caucasians. Recently, the publication of atomic structures of CFTR in two distinct conformations provides, for the first time, a clear overview of the protein. However, given the highly dynamic nature of the interactions among CFTR’s various domains, better understanding of the functional significance of these structures requires an integration of these new structural insights with previously established biochemical/biophysical studies, which is the goal of this review.
Structural mechanisms of CFTR function and dysfunction
J.-T. Yeh and J. Zhang contributed equally to this paper.
- Views Icon Views
- Share Icon Share
- Tools Icon Tools
- Search Site
Tzyh-Chang Hwang, Jiunn-Tyng Yeh, Jingyao Zhang, Ying-Chun Yu, Han-I Yeh, Samantha Destefano; Structural mechanisms of CFTR function and dysfunction. J Gen Physiol 2 April 2018; 150 (4): 539–570. doi: https://doi.org/10.1085/jgp.201711946
Download citation file: