Structural mechanisms of CFTR function and dysfunction

Tzyh Chang Hwang*, Jiunn Tyng Yeh, Jingyao Zhang, Ying Chun Yu, Han I. Yeh, Samantha Destefano

*此作品的通信作者

研究成果: Review article同行評審

61 引文 斯高帕斯(Scopus)

摘要

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.

原文English
頁(從 - 到)539-570
頁數32
期刊Journal of General Physiology
150
發行號4
DOIs
出版狀態Published - 1 4月 2018

指紋

深入研究「Structural mechanisms of CFTR function and dysfunction」主題。共同形成了獨特的指紋。

引用此