Review
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World J Biol Chem. Feb 26, 2014; 5(1): 26-39
Published online Feb 26, 2014. doi: 10.4331/wjbc.v5.i1.26
Cystic fibrosis transmembrane conductance regulator chloride channel blockers: Pharmacological, biophysical and physiological relevance
Paul Linsdell
Paul Linsdell, Department of Physiology and Biophysics, Dalhousie University, Halifax, Nova Scotia B3H 4R2, Canada
Author contributions: Linsdell P contributed to the manuscript.
Correspondence to: Paul Linsdell, PhD, Department of Physiology and Biophysics, Dalhousie University, PO Box 15000, Halifax, Nova Scotia B3H 4R2, Canada. paul.linsdell@dal.ca
Telephone: +1-902-4942265 Fax: +1-902-4941685
Received: October 2, 2013
Revised: November 15, 2013
Accepted: December 9, 2013
Published online: February 26, 2014
Core Tip

Core Tip: This review summarizes our understanding of small molecules that inhibit the cystic fibrosis transmembrane conductance regulator (CFTR) by blocking the channel pore. It describes how such inhibitors could be used in the treatment of diarrhea and hereditary kidney disease; how studying these inhibitors’ mechanisms of action has led to advances in our understanding of CFTR channel structure and function; and how substances acting via this mechanism could contribute to the physiological control of CFTR function in epithelial cells. Ironically, studying channel inhibitors has recently led to the discovery of a new class of CFTR potentiators that could be used to treat cystic fibrosis.