Cystic fibrosis affects about 1 in 2500 live births and involves loss of transmembrane chloride flux due to a lack of a membrane protein channel termed the cystic fibrosis transmembrane conductance regulator (CFTR). We have studied CFTR structure by electron crystallography. The data were compared with existing structures of other ATP-binding cassette transporters. The protein was crystallized ...

The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene was identified in 1989 by geneticist Lap-Chee Tsui and his research team as the gene associated with cystic fibrosis [4] (CF). Tsui?s research pinpointed the gene, some mutations to which cause CF, and it revealed the underlying disease mechanism. The CFTR gene encodes a protein in cell membranes in epithelial tissues and affec...

In the 8 yr since the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) was identified by means of positional cloning (Kerem et al., 1989; Riordan et al., 1989; Rommens et al., 1989), the Cl channel function of CFTR has been studied in a wide variety of expression systems. Macroscopic and single-channel currents have been measured and a large number of mutant construc...

The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene was identified in 1989 by geneticist Lap-Chee Tsui and his research team as the gene associated with cystic fibrosis [4] (CF). Tsui?s research pinpointed the gene, some mutations to which cause CF, and it revealed the underlying disease mechanism. The CFTR gene encodes a protein in cell membranes in epithelial tissues and affec...

Cystic fibrosis is a recessive autosomal disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. Cystic fibrosis-related diabetes (CFRD) is a common comorbidity of cystic fibrosis. Diabetic myonecrosis is a rare self-limited complication of poorly controlled diabetes mellitus that commonly presents with acute, intense pain and swelling of lower extremities a...

In this study, we examined whether mucociliary clearance differed between cystic fibrosis (CF) knockout mice and wildtype controls. Additionally, we investigated whether infection with Pseudomonas aeruginosa, a common pathogen in the CF lung, affected this important host defence mechanism. Ciliary beat frequency (fcb) and particle transport (PT) were recorded using an in vitro lung explant prep...

Cystic fibrosis (CF) is the most frequent life-limiting autosomal recessive disorder in Caucasian population. It due to mutations Fibrosis Transmembrane Conductance Regulator (CFTR) gene. Current symptomatic CF therapies, which treat downstream consequences of CFTR mutations, have increased survival. Better knowledge protein has enabled pharmacologic therapy aiming restore mutated expression an...

Cystic fibrosis (CF) is an autosomal recessive disorder that afflicts 72000 people world wide. It caused by over 2000 mutations to anion channel, the cystic transmembrane conductance regulator (CFtR) of which Over 300 present disease phenotype. We aim characterise all CF causing CFTR missense in Australian population using molecular dynamics modeling. Here we progress toward this goal presentin...