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Items: 1 to 20 of 814

1.
OTX2 regulates CFTR expression during endoderm differentiation and occupies 3′ cis-regulatory elements
Jenny L. Kerschner, Alekh Paranjapye, Monali NandyMazumdar, Shiyi Yin, Shih-Hsing Leir, Ann Harris
Dev Dyn. Author manuscript; available in PMC 2024 Jul 6.
Published in final edited form as: Dev Dyn. 2021 May; 250(5): 684–700. Published online 2021 Jan 26. doi: 10.1002/dvdy.293
PMCID:
PMC11227118
AbstractArticlePDF–2.2M
2.
Loss of CFTR Reverses Senescence Hallmarks in SARS-CoV-2 Infected Bronchial Epithelial Cells
Flavia Merigo, Anna Lagni, Federico Boschi, Paolo Bernardi, Anita Conti, Roberto Plebani, Mario Romano, Claudio Sorio, Virginia Lotti, Andrea Sbarbati
Int J Mol Sci. 2024 Jun; 25(11): 6185. Published online 2024 Jun 4. doi: 10.3390/ijms25116185
PMCID:
PMC11172982
AbstractArticlePDF–10M
3.
Prime editing functionally corrects cystic fibrosis-causing CFTR mutations in human organoids and airway epithelial cells
Mattijs Bulcaen, Phéline Kortleven, Ronald B. Liu, Giulia Maule, Elise Dreano, Mairead Kelly, Marjolein M. Ensinck, Sam Thierie, Maxime Smits, Matteo Ciciani, Aurelie Hatton, Benoit Chevalier, Anabela S. Ramalho, Xavier Casadevall i Solvas, Zeger Debyser, François Vermeulen, Rik Gijsbers, Isabelle Sermet-Gaudelus, Anna Cereseto, Marianne S. Carlon
Cell Rep Med. 2024 May 21; 5(5): 101544. Published online 2024 May 1. doi: 10.1016/j.xcrm.2024.101544
PMCID:
PMC11148721
AbstractArticlePDF–5.1M
4.
Insights and considerations on CFTR variant reporting in a study of cystic fibrosis patients in Saudi Arabia CFTR 1548del G and 1549del G: Navigating the discovery of novel mutations
Eman A. Al Abdulsalam
Saudi Med J. 2024 May; 45(5): 541. doi: 10.15537/smj.2024.45.5.20240331
PMCID:
PMC11147566
ArticlePDF–112K
5.
Nucleotide binding domains of human CFTR: a structural classification of critical residues and disease-causing mutations.
R. Eudes, P. Lehn, C. Férec, J. -P. Mornon, I. Callebaut
Cell Mol Life Sci. 2005 Sep; 62(18): 2112–2123. Published online 2005 Aug 26. doi: 10.1007/s00018-005-5224-y
PMCID:
PMC11139114
AbstractArticlePDF–742K
6.
Atomic model of human cystic fibrosis transmembrane conductance regulator: Membrane-spanning domains and coupling interfaces
J.-P. Mornon, P. Lehn, I. Callebaut
Cell Mol Life Sci. 2008 Aug; 65(16): 2594–2612. Published online 2008 Jul 4. doi: 10.1007/s00018-008-8249-1
PMCID:
PMC11131860
AbstractArticlePDF–5.3M
7.
Molecular models of the open and closed states of the whole human CFTR protein
Jean-Paul Mornon, Pierre Lehn, Isabelle Callebaut
Cell Mol Life Sci. 2009 Nov; 66(21): 3469–3486. Published online 2009 Aug 26. doi: 10.1007/s00018-009-0133-0
PMCID:
PMC11115851
AbstractArticlePDF–1.1M
8.
Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics
Jean-Paul Mornon, Brice Hoffmann, Slavica Jonic, Pierre Lehn, Isabelle Callebaut
Cell Mol Life Sci. 2015 Apr; 72(7): 1377–1403. Published online 2014 Oct 7. doi: 10.1007/s00018-014-1749-2
PMCID:
PMC11113974
AbstractArticlePDF–7.6M
9.
Functional and pharmacological induced structural changes of the cystic fibrosis transmembrane conductance regulator in the membrane solved using SAXS
Debora Baroni, Olga Zegarra-Moran, Oscar Moran
Cell Mol Life Sci. 2015 Apr; 72(7): 1363–1375. Published online 2014 Oct 2. doi: 10.1007/s00018-014-1747-4
PMCID:
PMC11113906
AbstractArticlePDF–2.0M
10.
A SAXS-based ensemble model of the native and phosphorylated regulatory domain of the CFTR
Carlotta Marasini, Lauretta Galeno, Oscar Moran
Cell Mol Life Sci. 2013 Mar; 70(5): 923–933. Published online 2012 Oct 4. doi: 10.1007/s00018-012-1172-5
PMCID:
PMC11113146
AbstractArticlePDF–532K
11.
From the endoplasmic reticulum to the plasma membrane: mechanisms of CFTR folding and trafficking
Carlos M. Farinha, Sara Canato
Cell Mol Life Sci. 2017 Jan; 74(1): 39–55. Published online 2016 Oct 3. doi: 10.1007/s00018-016-2387-7
PMCID:
PMC11107782
AbstractArticlePDF–1.6M
12.
The biophysics, biochemistry and physiology of CFTR
Oscar Moran
Cell Mol Life Sci. 2017 Jan; 74(1): 1–2. Published online 2016 Oct 4. doi: 10.1007/s00018-016-2384-x
PMCID:
PMC11107758
ArticlePDF–302K
13.
The gating of the CFTR channel
Oscar Moran
Cell Mol Life Sci. 2017 Jan; 74(1): 85–92. Published online 2016 Oct 1. doi: 10.1007/s00018-016-2390-z
PMCID:
PMC11107742
AbstractArticlePDF–968K
14.
Current insights into the role of PKA phosphorylation in CFTR channel activity and the pharmacological rescue of cystic fibrosis disease-causing mutants
Stephanie Chin, Maurita Hung, Christine E. Bear
Cell Mol Life Sci. 2017 Jan; 74(1): 57–66. Published online 2016 Oct 8. doi: 10.1007/s00018-016-2388-6
PMCID:
PMC11107731
AbstractArticlePDF–793K
15.
Molecular modelling and molecular dynamics of CFTR
Isabelle Callebaut, Brice Hoffmann, Pierre Lehn, Jean-Paul Mornon
Cell Mol Life Sci. 2017 Jan; 74(1): 3–22. Published online 2016 Oct 7. doi: 10.1007/s00018-016-2385-9
PMCID:
PMC11107702
AbstractArticlePDF–3.9M
16.
Architecture and functional properties of the CFTR channel pore
Paul Linsdell
Cell Mol Life Sci. 2017 Jan; 74(1): 67–83. Published online 2016 Oct 3. doi: 10.1007/s00018-016-2389-5
PMCID:
PMC11107662
AbstractArticlePDF–4.5M
17.
Conformational change of the extracellular parts of the CFTR protein during channel gating
Alexander Negoda, Elizabeth A. Cowley, Yassine El Hiani, Paul Linsdell
Cell Mol Life Sci. 2018 Aug; 75(16): 3027–3038. Published online 2018 Feb 14. doi: 10.1007/s00018-018-2777-0
PMCID:
PMC11105745
AbstractArticlePDF–1.7M
18.
Cystic fibrosis transmembrane conductance regulator—emerging regulator of cancer
Jieting Zhang, Yan Wang, Xiaohua Jiang, Hsiao Chang Chan
Cell Mol Life Sci. 2018 May; 75(10): 1737–1756. Published online 2018 Feb 6. doi: 10.1007/s00018-018-2755-6
PMCID:
PMC11105598
AbstractArticlePDF–1.3M
19.
Proteomic interaction profiling reveals KIFC1 as a factor involved in early targeting of F508del-CFTR to degradation
Sara Canato, João D. Santos, Ana S. Carvalho, Kerman Aloria, Margarida D. Amaral, Rune Matthiesen, André O. Falcao, Carlos M. Farinha
Cell Mol Life Sci. 2018 Dec; 75(24): 4495–4509. Published online 2018 Jul 31. doi: 10.1007/s00018-018-2896-7
PMCID:
PMC11105581
AbstractArticlePDF–1.9M
20.
Impairment of CFTR activity in cultured epithelial cells upregulates the expression and activity of LDH resulting in lactic acid hypersecretion
Ángel G. Valdivieso, Mariángeles Clauzure, María M. Massip-Copiz, Carla E. Cancio, Cristian J. A. Asensio, Consuelo Mori, Tomás A. Santa-Coloma
Cell Mol Life Sci. 2019 Apr; 76(8): 1579–1593. Published online 2019 Jan 1. doi: 10.1007/s00018-018-3001-y
PMCID:
PMC11105536
AbstractArticlePDF–3.4M

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