The increase in numbers of adults with cystic fibrosis (CF) has allowed us to identify previously unrecognized chronic complications of CF, as well as appreciate unique presentations of cystic fibrosis-related diseases. Do these chronic complications and unique presentations provide us with new insight into cystic fibrosis transmembrane conductance regulator (CFTR) function? Current data sugges...

This review summarizes the newly discovered molecular mechanism of secretin-stimulated urine HCO3– excretion and role cystic fibrosis transmembrane conductance regulator (CFTR) in renal excretion. The secretin receptor is functionally expressed basolateral membrane HCO3–-secreting β-intercalated cells collecting duct. Here it activates a fast efficient secretion into serving to normalize metabo...

  Cystic fibrosis (CF) is a life-limiting autosomal recessive disorder affecting principally respiratory and digestive system . It is caused by cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation. The aim of this study was to determine the extent of repeat numbers and the degree of heterozygosity for c.3499+200TA(7_56) and D7S523 located in intron 17b and 1 cM proximal to t...

The cystic fibrosis transmembrane conductance regulator (CFTR) has been known for the past 11 years to be a membrane glycoprotein with chloride channel activity. Only recently has the glycosylation of CFTR been examined in detail, by O'Riordan et al in Glycobiology. Using cells that overexpress wild-type (wt)CFTR, the presence of polylactosamine was noted on the fully glycosylated form of CFTR....

Regulator (ABCC7) Structure Cystic Fibrosis Transmembrane Conductance John F. Hunt, Chi Wang and Robert C. Ford Perspective The Cystic Fibrosis Gene: A Molecular Genetic Lap-Chee Tsui and Ruslan Dorfman Cystic Fibrosis Status of Fluid and Electrolyte Absorption in M.M. Reddy and M. Jackson Stutts Anion Permeation The CFTR Ion Channel: Gating, Regulation, and Tzyh-Chang Hwang and Kevin L. Kirk P...

Cystic fibrosis results from defects in the gene encoding a cyclic adenosine monophosphate-dependent chloride ion channel known as the cystic fibrosis transmembrane conductance regulator (CFTR). To create an animal model for cystic fibrosis, mice were generated from embryonic stem cells in which the CFTR gene was disrupted by gene targeting. Mice homozygous for the disrupted gene display many f...

The epithelial response to bacterial airway infection, a common feature of lung diseases such as chronic obstructive pulmonary disease and cystic fibrosis, has been extensively studied. However, its impact on cystic fibrosis transmembrane conductance regulator (CFTR) channel function is not clearly defined. Our aims were, therefore, to evaluate the effect of Pseudomonas aeruginosa on CFTR funct...

cystic fibrosis (cf) is a life-limiting autosomal recessive disorder affecting principally respiratory and digestive system . it is caused by cystic fibrosis transmembrane conductance regulator (cftr) gene mutation. the aim of this study was to determine the extent of repeat numbers and the degree of heterozygosity for c.3499+200ta(7_56) and d7s523 located in intron 17b and 1 cm proximal to the...

Curcumin has been reported to correct cystic fibrosis caused by the DeltaF508 mutation of the cystic fibrosis transmembrane regulator (CFTR) but its mechanistic action remains unclear. We have recently demonstrated that the ER chaperone calreticulin (CRT) negatively regulates the CFTR cell surface expression and activity. Thus, we aimed at determining whether CRT mediates the effect of curcumin...

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...