... uncertain. The cystic fibrosis transmembrane conductance regulator (CFTR), which is mutated in cystic fibrosis (CF), forms clusters that are cholesterol dependent and become incorporated into long-lived platforms during hormonal stimulation. We report here that clustering does not involve known tethering...

...Anna C. Abbattiscianni; Maria Favia; Maria T. Mancini; Rosa A. Cardone; Lorenzo Guerra; Stefania Monterisi; Stefano Castellani; Onofrio Laselva; Francesca Di Sole; Massimo Conese; Manuela Zaccolo; Valeria Casavola ABSTRACT The most common mutation of the cystic fibrosis transmembrane regulator...

...Stefania Monterisi; Maria Favia; Lorenzo Guerra; Rosa A. Cardone; Domenico Marzulli; Stephan J. Reshkin; Valeria Casavola; Manuela Zaccolo The cystic fibrosis transmembrane conductance regulator (CFTR) mutation ΔF508CFTR still causes regulatory defects when rescued to the apical membrane...

...-terminal PDZ motif of MUC3, binding to the single GOPC PDZ domain. GOPC is also a binding partner for cystic fibrosis transmembrane conductance regulator (CFTR) that directs CFTR for degradation. Overexpression of GOPC downregulated the total levels of MUC3, an effect that was reversed by introducing CFTR...

...Martina Gentzsch; Amit Choudhury; Xiu-bao Chang; Richard E. Pagano; John R. Riordan Most patients with cystic fibrosis (CF) have a single codon deletion (ΔF508) in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) that impairs assembly of the multidomain glycoprotein...

...Ling Yang; Jeff Reece; Sherif E. Gabriel; Stephen B. Shears A new aspect of research into the pathogenesis of cystic fibrosis (CF) is a genetics-based search for `modifier genes' that may affect the severity of CF lung disease. Using an alternative, cell biological approach, we show that ITPK1...

...-associated degradation Polytopic proteins Cystic fibrosis ER dislocation Proteasome inhibitors p97 Membrane proteins in the endoplasmic reticulum (ER) that fail to acquire proper tertiary or quaternary structure are recognized by ER quality control machinery and degraded by the ubiquitin...

.... Prior studies have shown that the cytoplasmic carboxyl terminus of the cystic fibrosis transmembrane conductance regulator (CFTR) is involved in the apical localization of this protein. Here we show that the C-terminal tail alone, or when fused to the green fluorescent protein (GFP), can localize...

...Lynda S. Ostedgaard; Bernhardt Zeiher; Michael J. Welsh ABSTRACT Cystic fibrosis transmembrane conductance regulator (CFTR) containing the ΔF508 mutation is retained in the endoplasmic reticulum (ER). This defect can be partially overcome by a reduction in temperature which allows some of the ΔF508...

... in response to elevations in intracellular cAMP. * Author for correspondence (e-mail: benos@phybio.bhs.uab.edu ) 28 08 1995 16 03 1996 © 1996 by Company of Biologists 1996 Chloride channel Cystic fibrosis Colonic epithelia The acute recruitment of transport proteins...

...Jenny Walker; Judy Watson; Christopher Holmes; Aleksander Edelman; George Banting ABSTRACT We have raised mouse monoclonal antibodies to eight synthetic peptides corresponding to different regions of the human cystic fibrosis transmembrane conductance regulator (CFTR) and rabbit polyclonal antisera...

... by the presence of multiple second messengers (other than cAMP), multiple intracellular pools of calcium, or cell heterogeneity. The agonists tested had the same relative potency in cells from patients with cystic fibrosis as in non-cystic fibrosis cells. * Author for correspondence 25 11 1994 08...

...Michael J. Welsh; Gerene M. Denning; Lynda S. Ostedgaard; Matthew P. Anderson SUMMARY The cystic fibrosis transmembrane conductance regulator (CFTR) is mutated in patients with cystic fibrosis (CF). The most common CF-associated mutation is deletion of phenylanine at residue 508, CFTRΔF508. When...

...Jonathan Barasch; Qais Al-Awqati SUMMARY Cystic fibrosis is associated with defective epithelial sodium chloride and fluid secretion in epithelia. In addition, there is widespread reductions in sialylation of secreted proteins and increases in the sulfation and fucosylation of mucus glycoproteins...

...Lindsay Coleman; Ann Harris ABSTRACT The epithelia lining the vas deferens and epididymis are directly involved in the pathology of the autosomal recessive disease cystic fibrosis (CF). We have established culture systems for these epithelial cells. Long-term cell lines have now been generated from...

...Janet A. Buchanan; Herman Yeger; Joseph A. Tabcharani; Timothy J. Jensen; Wojtek Auerbach; John W. Hanrahan; John R. Riodan; Manuel Buchwald ABSTRACT We undertook to extend the in vitro lifespan of epithelial cell cultures useful for the study of the cellular defect underlying cystic fibrosis (CF...

... for considerable periods of time. These cells will provide a suitable System for investigating, by electrophysiological, biochemical and molecular biological methods, the cause of sterility in cystic fibrosis. 06 12 1989 16 01 1989 © 1989 by Company of Biologists 1989 vas deferens...

...Maureen M. Wilkinson; A. Busuttil; Caroline Hayward; D. J. H. Brock; Julia R. Dorin; Veronica Van Heyningen ABSTRACT This paper reports further study of the identity and function of a protein shown to be elevated in serum from cystic fibrosis (CF) patients and clinically normal heterozygotes...

... cell types within the intact foetal pancreas. Results support the view that the cultured cells are ductal in origin and nature. Pancreatic epithelial cell cultures have also been established from foetuses with cystic fibrosis. © 1988 by Company of Biologists 1988 pancreas ductal epithelial...

... of the view that these cells are ductal in origin. They are likely to be one of the few cell types that express the basic defect of cystic fibrosis in vitro . The cells may be passaged and sufficient material obtained to permit biochemical and molecular biological analysis. 05 02 1987 23 03 1987...