...Aurélien Bidaud-Meynard; Florian Bossard; Andrea Schnúr; Ryosuke Fukuda; Guido Veit; Haijin Xu; Gergely L. Lukacs ABSTRACT Apical polarity of cystic fibrosis transmembrane conductance regulator (CFTR) is essential for solute and water transport in secretory epithelia and can be impaired in human...

... to regulate CFTR channel gating – the protein defective in cystic fibrosis – the contribution of EPAC1 to CFTR regulation remains largely undefined. Here, we demonstrate that in human airway epithelial cells, cAMP signaling through EPAC1 promotes CFTR stabilization at the plasma membrane by attenuating its...

...Ye Chun Ruan; Yan Wang; Nicolas Da Silva; Bongki Kim; Rui Ying Diao; Eric Hill; Dennis Brown; Hsiao Chang Chan; Sylvie Breton ABSTRACT Mutations in CFTR lead to dysfunction of tubular organs, which is currently attributed to impairment of its conductive properties. We now show that CFTR regulates...

...John P. Holleran; Jianxin Zeng; Raymond A. Frizzell; Simon C. Watkins Summary Efficient trafficking of the cystic fibrosis transmembrane conductance regulator (CFTR) to and from the cell surface is essential for maintaining channel density at the plasma membrane (PM) and ensuring proper...

... with green fluorescent protein in their acrosomes, it was shown that the lower [Na + ] i concentration only occurs in sperm having intact acrosomes. ENaC inhibition has been shown in other cell types to depend on the activation of cystic fibrosis transmembrane conductance regulator (CFTR). In non-capacitated...

.... GPIAPs, such as Thy-1, require clustering, cholesterol and Src-family kinase (SFK) activity to become transiently anchored. By contrast, a transmembrane protein, the cystic fibrosis transmembrane conductance regulator (CFTR), which has a PDZ-binding motif in its cytoplasmic C-terminus that binds the ERM...

...Xiu-bao Chang; April Mengos; Yue-xian Hou; Liying Cui; Timothy J. Jensen; Andrei Aleksandrov; John R. Riordan; Martina Gentzsch The epithelial chloride channel CFTR is a glycoprotein that is modified by two N-linked oligosaccharides. The most common mutant CFTR protein in patients with cystic...

...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...

... Donselaar, E., Posthuma, G., de Jonge, H., McLaughlin, G., Geuze, H. J., Marino, C. and Peters, P. J. ( 2000 ). Subcellular distribution of CFTR in rat intestine supports a physiologic role for CFTR regulation by vesicle traffic. Histochem. Cell Biol. 114 , 219 -228. Berridge, M. J., Lipp, P...

...Jon Oberdorf; Eric J. Carlson; William R. Skach The 26S proteasome is the primary protease responsible for degrading misfolded membrane proteins in the endoplasmic reticulum. Here we examine the specific role of β subunit function on polypeptide cleavage and membrane release of CFTR, a prototypical...

...Frédéric Bilan; Vincent Thoreau; Magali Nacfer; Renaud Dérand; Caroline Norez; Anne Cantereau; Martine Garcia; Frédéric Becq; Alain Kitzis The cystic fibrosis transmembrane conductance regulator (CFTR) is a cyclic AMP-dependent chloride channel that mediates electrolyte transport across...

... transmembrane conductance regulator (CFTR) delF508 channels in IB3-1 human cells, which express endogenous levels of delF508-CFTR. These drugs were without effect on the Ca 2+ -activated Cl – transport, whereas the swelling-activated Cl – transport was found altered in MPB-treated cells. Immunoprecipitation...

.... 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...

... polarized epithelial cells. * Author for correspondence (e-mail: [email protected] ) 28 11 2000 © 2001 by Company of Biologists 2001 Cell polarity Intestinal enzymes CFTR Keratin knockout Microvilli γ-Tubulin Microtubules Syntaxin 3 Bile canaliculus Enterocytes...

...Nadia A. Ameen; Birgitta Martensson; Lilly Bourguinon; Christopher Marino; Jon Isenberg; Gwenn E. McLaughlin ABSTRACT cAMP activated insertion of the cystic fibrosis transmembrane conductance regulator (CFTR) channels from endosomes to the apical plasma membrane has been hypothesized to regulate...

...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...

...Julie A. Chambers; Ann Harris ABSTRACT The main pathology of cystic fibrosis results from obstruction of ducts in several organs by mucous secretions. The cause of this obstruction remains unclear. We have examined expression of the cystic fibrosis transmembrane conductance regulator (CFTR...