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Select item 112271181.

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

Abstract Article PDF–2.2M

Select item 111729822.

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

Abstract Article PDF–10M

Select item 111487213.

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

Abstract Article PDF–5.1M

Select item 111475664.

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

Article PDF–112K

Select item 111391145.

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

Abstract Article PDF–742K

Select item 111318606.

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

Abstract Article PDF–5.3M

Select item 111158517.

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

Abstract Article PDF–1.1M

Select item 111139748.

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

Abstract Article PDF–7.6M

Select item 111139069.

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

Abstract Article PDF–2.0M

Select item 1111314610.

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

Abstract Article PDF–532K

Select item 1110778211.

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

Abstract Article PDF–1.6M

Select item 1110775812.

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

Article PDF–302K

Select item 1110774213.

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

Abstract Article PDF–968K

Select item 1110773114.

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

Abstract Article PDF–793K

Select item 1110770215.

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

Abstract Article PDF–3.9M

Select item 1110766216.

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

Abstract Article PDF–4.5M

Select item 1110574517.

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

Abstract Article PDF–1.7M

Select item 1110559818.

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

Abstract Article PDF–1.3M

Select item 1110558119.

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

Abstract Article PDF–1.9M

Select item 1110553620.

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

Abstract Article PDF–3.4M

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