On the mechanism of gating defects caused by the R117H mutation in cystic fibrosis transmembrane conductance regulator.

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The most common cystic fibrosis-associated mutation destabilizes the dimeric state of the nucleotide-binding domains of CFTR. J Physiol (2011) 1.04

Changes in accessibility of cytoplasmic substances to the pore associated with activation of the cystic fibrosis transmembrane conductance regulator chloride channel. J Biol Chem (2010) 1.03

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Three-dimensional reconstruction of human cystic fibrosis transmembrane conductance regulator chloride channel revealed an ellipsoidal structure with orifices beneath the putative transmembrane domain. J Biol Chem (2008) 0.97

The silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunction. FASEB J (2013) 0.93

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Three charged amino acids in extracellular loop 1 are involved in maintaining the outer pore architecture of CFTR. J Gen Physiol (2014) 0.86

Modulation of CFTR gating by permeant ions. J Gen Physiol (2014) 0.86

Conformational changes in the catalytically inactive nucleotide-binding site of CFTR. J Gen Physiol (2013) 0.84

Disease-associated mutations in the extracytoplasmic loops of cystic fibrosis transmembrane conductance regulator do not impede biosynthetic processing but impair chloride channel stability. J Biol Chem (2001) 0.83