scholarly journals Nonequilibrium Gating of CFTR on an Equilibrium Theme

Physiology ◽  
2012 ◽  
Vol 27 (6) ◽  
pp. 351-361 ◽  
Author(s):  
Kang-Yang Jih ◽  
Tzyh-Chang Hwang
Keyword(s):  
Cystic Fibrosis ◽  
Abc Transporter ◽  
Chloride Channel ◽  
Genetic Disease ◽  
Clinical Relevance ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Abc Protein ◽  
Gating Mechanism ◽  
Cystic Fibrosis Transmembrane

Malfunction of cystic fibrosis transmembrane conductance regulator (CFTR), a member of the ABC protein superfamily that functions as an ATP-gated chloride channel, causes the lethal genetic disease, cystic fibrosis. This review focuses on the most recent findings on the gating mechanism of CFTR. Potential clinical relevance and implications to ABC transporter function are also discussed.

scholarly journals Exploiting species differences to understand the CFTR Cl− channel

2015 ◽  
Vol 43 (5) ◽  
pp. 975-982 ◽  
Author(s):  
Samuel J. Bose ◽  
Toby S. Scott-Ward ◽  
Zhiwei Cai ◽  
David N. Sheppard
Keyword(s):  
Cystic Fibrosis ◽  
Phylogenetic Analysis ◽  
Abc Transporter ◽  
Genetic Disease ◽  
Species Differences ◽  
Anion Channel ◽  
Transmembrane Conductance Regulator ◽  
Transmembrane Conductance ◽  
Aquatic Vertebrates ◽  
Cystic Fibrosis Transmembrane

The anion channel cystic fibrosis transmembrane conductance regulator (CFTR) is a unique ATP-binding cassette (ABC) transporter. CFTR plays a pivotal role in transepithelial ion transport as its dysfunction in the genetic disease cystic fibrosis (CF) dramatically demonstrates. Phylogenetic analysis suggests that CFTR first appeared in aquatic vertebrates fulfilling important roles in osmosensing and organ development. Here, we review selectively, knowledge of CFTR structure, function and pharmacology, gleaned from cross-species comparative studies of recombinant CFTR proteins, including CFTR chimeras. The data argue that subtle changes in CFTR structure can affect strongly channel function and the action of CF mutations.

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