Sheppard, David N., and Michael J. Welsh. Structure and Function of the CFTR Chloride
Channel. Physiol. Rev. 79, Suppl.: S23–S45, 1999.—The cystic fibrosis transmembrane …

Cystic fibrosis is a recessive disorder common among Caucasians. Although the disease
has been known in the 1930s, it was only in the late 1980s that the molecular mechanisms …

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) gene which encodes a protein expressed in the apical membrane of exocrine …

Publisher Summary The functional properties of ion channels, such as gating, ion selectivity,
single-channel conductance, multi-ion occupancy, transient blocking, desensitization, and …

We have probed internal and external accessibility of S4 residues to the membrane-
impermeant thiol reagent methanethiosulfonate-ethyltrimethylammonium (MTSET) in both …

Thermodynamic mutant cycles provide a formalism for studying energetic coupling between
amino acids on the interaction surface in a protein-protein complex. This approach was …

Proteins that form aqueous channels in membranes generate conduction pathways with a
variety of shapes and sizes. Perhaps the largest channel-forming protein is the 2-MDa …

Defective epithelial ion transport is the hallmark of the life-limiting genetic disease cystic
fibrosis (CF). This abnormality is caused by mutations in the cystic fibrosis transmembrane …

Integral membrane proteins are found in all cellular membranes and carry out many of the
functions that are essential to life. The membrane-embedded domains of integral membrane …

The cystic fibrosis transmembrane conductance regulator (CFTR) 1 forms a Cl channel that
is an essential component of epithelial Cl transport systems in many organs, including the …