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Keywords: CFTR
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Journal Articles
J Cell Sci (2019) 132 (10): jcs226886.
Published: 15 May 2019
...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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2016) 129 (13): 2599–2612.
Published: 01 July 2016
... 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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2014) 127 (20): 4396–4408.
Published: 15 October 2014
...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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2013) 126 (12): 2692–2703.
Published: 15 June 2013
...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...
Includes: Supplementary data
Journal Articles
J Cell Sci (2012) 125 (2): 473–485.
Published: 15 January 2012
... 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...
Includes: Supplementary data
Journal Articles
In collection:
Lipid Biology
J Cell Sci (2009) 122 (21): 3966–3972.
Published: 01 November 2009
.... 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...
Includes: Multimedia, Supplementary data
Journal Articles
J Cell Sci (2008) 121 (17): 2814–2823.
Published: 01 September 2008
...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...
Includes: Supplementary data
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
... (email: shears@niehs.nih.gov ) 14 12 2005 © The Company of Biologists Limited 2006 2006 ITPK1 Cystic fibrosis inositol phosphates CFTR Secretion Chloride channel Individuals that suffer from cystic fibrosis (CF) have an average life-span of just over 30 years, and almost...
Journal Articles
J Cell Sci (2006) 119 (2): 303–313.
Published: 15 January 2006
...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...
Journal Articles
J Cell Sci (2004) 117 (10): 1923–1935.
Published: 15 April 2004
...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...
Journal Articles
J Cell Sci (2001) 114 (22): 4073–4081.
Published: 15 November 2001
... 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...