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Keywords: Cystic fibrosis
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Journal Articles
J Cell Sci jcs.259002.
Published: 21 January 2022
.... 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 interactions...
Journal Articles
J Cell Sci (2016) 129 (6): 1128–1140.
Published: 15 March 2016
...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...
Journal Articles
J Cell Sci (2012) 125 (5): 1106–1117.
Published: 01 March 2012
...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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2011) 124 (18): 3074–3083.
Published: 15 September 2011
...-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...
Journal Articles
J Cell Sci (2007) 120 (3): 447–455.
Published: 01 February 2007
...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...
Journal Articles
J Cell Sci (2006) 119 (7): 1320–1328.
Published: 01 April 2006
...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...
Journal Articles
J Cell Sci (2006) 119 (2): 303–313.
Published: 15 January 2006
..., 3710 SW US Veterans Hospital Road, Portland, OR 97239, USA 5 10 2005 © The Company of Biologists Limited 2006 2006 CFTR ER-associated degradation Polytopic proteins Cystic fibrosis ER dislocation Proteasome inhibitors p97 Membrane proteins in the endoplasmic reticulum...
Journal Articles
J Cell Sci (1993) 1993 (Supplement_17): 235–239.
Published: 01 December 1993
...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...
Journal Articles
J Cell Sci (1993) 1993 (Supplement_17): 229–235.
Published: 01 December 1993
...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...