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 the most common potentially lethal autosomal recessive disease of
Caucasians, affecting 1 in 2500 newborns. Since the recent identification of the gene that is …

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 …

Circumstantial evidence has accumulated suggesting that CFTR is a regulated low-
conductance Cl-channel. To test this postulate directly, we have purified to homogeneity a …

The cystic fibrosis transmembrane conductance regulator (CFTR) acts as a channel on the
apical membrane of epithelia. Disease-causing mutations in the cystic fibrosis gene can …

The cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel
evolved from an ATP-binding cassette transporter. CFTR channel gating is strictly coupled to …

Adenosine deaminases that act on RNA are a conserved family of enzymes that catalyze a
natural process of site-directed mutagenesis. Biochemically, they convert adenosine to …

Deletion of the phenylalanine at position 508 of the cystic fibrosis transmembrane
conductance regulator (CFTR) is the most prevalent mutation in cystic fibrosis (CF). This …

Direct transfer of the normal cystic fibrosis (CF) transmembrane conductance regulator
(CFTR) gene to airway epitheiium was evaluated using a replicationdeficient recombinant …

Variable in–frame skipping of exon 9 in cystic fibrosis transmembrane conductance
regulator (CFTR) mRNA transcripts (exon 9−) occurs in the respiratory epithelium. To …