Literature DB >> 30287473

The cystic fibrosis airway milieu enhances rescue of F508del in a pre-clinical model.

Martina Gentzsch1,2, Deborah M Cholon1, Nancy L Quinney1, Susan E Boyles1, Mary E B Martino1, Carla M P Ribeiro1,2,3.   

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Year:  2018        PMID： 30287473      PMCID： PMC6482470          DOI： 10.1183/13993003.01133-2018

Source DB:  PubMed          Journal:  Eur Respir J        ISSN： 0903-1936            Impact factor:   16.671


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  15 in total

1.  Restoration of R117H CFTR folding and function in human airway cells through combination treatment with VX-809 and VX-770.

Authors:  Martina Gentzsch; Hong Y Ren; Scott A Houck; Nancy L Quinney; Deborah M Cholon; Pattarawut Sopha; Imron G Chaudhry; Jhuma Das; Nikolay V Dokholyan; Scott H Randell; Douglas M Cyr
Journal:  Am J Physiol Lung Cell Mol Physiol       Date:  2016-07-08       Impact factor: 5.464

2.  Efficacy of lumacaftor-ivacaftor for the treatment of cystic fibrosis patients homozygous for the F508del-CFTR mutation.

Authors:  Deborah M Cholon; Charles R Esther; Martina Gentzsch
Journal:  Expert Rev Precis Med Drug Dev       Date:  2016-04-22

3.  Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del.

Authors:  Jennifer L Taylor-Cousar; Anne Munck; Edward F McKone; Cornelis K van der Ent; Alexander Moeller; Christopher Simard; Linda T Wang; Edward P Ingenito; Charlotte McKee; Yimeng Lu; Julie Lekstrom-Himes; J Stuart Elborn
Journal:  N Engl J Med       Date:  2017-11-03       Impact factor: 91.245

4.  Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR.

Authors:  Claire E Wainwright; J Stuart Elborn; Bonnie W Ramsey; Gautham Marigowda; Xiaohong Huang; Marco Cipolli; Carla Colombo; Jane C Davies; Kris De Boeck; Patrick A Flume; Michael W Konstan; Susanna A McColley; Karen McCoy; Edward F McKone; Anne Munck; Felix Ratjen; Steven M Rowe; David Waltz; Michael P Boyle
Journal:  N Engl J Med       Date:  2015-05-17       Impact factor: 91.245

5.  Chronic airway infection/inflammation induces a Ca2+i-dependent hyperinflammatory response in human cystic fibrosis airway epithelia.

Authors:  Carla M Pedrosa Ribeiro; Anthony M Paradiso; Ute Schwab; Juan Perez-Vilar; Lisa Jones; Wanda O'neal; Richard C Boucher
Journal:  J Biol Chem       Date:  2005-03-03       Impact factor: 5.157

6.  Airway epithelial inflammation-induced endoplasmic reticulum Ca2+ store expansion is mediated by X-box binding protein-1.

Authors:  Mary E B Martino; John C Olsen; Nanette B Fulcher; Matthew C Wolfgang; Wanda K O'Neal; Carla M P Ribeiro
Journal:  J Biol Chem       Date:  2009-03-25       Impact factor: 5.157

7.  A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial.

Authors:  Michael P Boyle; Scott C Bell; Michael W Konstan; Susanna A McColley; Steven M Rowe; Ernst Rietschel; Xiaohong Huang; David Waltz; Naimish R Patel; David Rodman
Journal:  Lancet Respir Med       Date:  2014-06-24       Impact factor: 30.700

8.  Mucin Production and Hydration Responses to Mucopurulent Materials in Normal versus Cystic Fibrosis Airway Epithelia.

Authors:  Lubna H Abdullah; Raymond Coakley; Megan J Webster; Yunxiang Zhu; Robert Tarran; Giorgia Radicioni; Mehmet Kesimer; Richard C Boucher; C William Davis; Carla M P Ribeiro
Journal:  Am J Respir Crit Care Med       Date:  2018-02-15       Impact factor: 30.528

9.  Pseudomonas aeruginosa Reduces VX-809 Stimulated F508del-CFTR Chloride Secretion by Airway Epithelial Cells.

Authors:  Bruce A Stanton; Bonita Coutermarsh; Roxanna Barnaby; Deborah Hogan
Journal:  PLoS One       Date:  2015-05-27       Impact factor: 3.240

10.  Azithromycin treatment alters gene expression in inflammatory, lipid metabolism, and cell cycle pathways in well-differentiated human airway epithelia.

Authors:  Carla Maria P Ribeiro; Harry Hurd; Yichao Wu; Mary E B Martino; Lisa Jones; Brian Brighton; Richard C Boucher; Wanda K O'Neal
Journal:  PLoS One       Date:  2009-06-05       Impact factor: 3.240
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  7 in total

Review 1.  Changes in the Cystic Fibrosis Airway Microbiome in Response to CFTR Modulator Therapy.

Authors:  Buqing Yi; Alexander H Dalpke; Sébastien Boutin
Journal:  Front Cell Infect Microbiol       Date:  2021-03-17       Impact factor: 5.293

2.  Airway Epithelial Inflammation In Vitro Augments the Rescue of Mutant CFTR by Current CFTR Modulator Therapies.

Authors:  Martina Gentzsch; Deborah M Cholon; Nancy L Quinney; Mary E B Martino; John T Minges; Susan E Boyles; Tara N Guhr Lee; Charles R Esther; Carla M P Ribeiro
Journal:  Front Pharmacol       Date:  2021-03-30       Impact factor: 5.810

3.  Culture with apically applied healthy or disease sputum alters the airway surface liquid proteome and ion transport across human bronchial epithelial cells.

Authors:  Maximillian Woodall; Boris Reidel; Mehmet Kesimer; Robert Tarran; Deborah L Baines
Journal:  Am J Physiol Cell Physiol       Date:  2021-10-06       Impact factor: 4.249

Review 4.  Impact of Airway Inflammation on the Efficacy of CFTR Modulators.

Authors:  Carla M P Ribeiro; Martina Gentzsch
Journal:  Cells       Date:  2021-11-22       Impact factor: 6.600

Review 5.  CFTR Modulator Therapies: Potential Impact on Airway Infections in Cystic Fibrosis.

Authors:  Francesca Saluzzo; Luca Riberi; Barbara Messore; Nicola Ivan Loré; Irene Esposito; Elisabetta Bignamini; Virginia De Rose
Journal:  Cells       Date:  2022-04-06       Impact factor: 6.600

6.  Measuring cystic fibrosis drug responses in organoids derived from 2D differentiated nasal epithelia.

Authors:  Gimano D Amatngalim; Lisa W Rodenburg; Bente L Aalbers; Henriette Hm Raeven; Ellen M Aarts; Dounia Sarhane; Sacha Spelier; Juliet W Lefferts; Iris Al Silva; Wilco Nijenhuis; Sacha Vrendenbarg; Evelien Kruisselbrink; Jesse E Brunsveld; Cornelis M van Drunen; Sabine Michel; Karin M de Winter-de Groot; Harry G Heijerman; Lukas C Kapitein; Magarida D Amaral; Cornelis K van der Ent; Jeffrey M Beekman
Journal:  Life Sci Alliance       Date:  2022-08-03

Review 7.  CFTR Modulators: The Changing Face of Cystic Fibrosis in the Era of Precision Medicine.

Authors:  Miquéias Lopes-Pacheco
Journal:  Front Pharmacol       Date:  2020-02-21       Impact factor: 5.810
  7 in total

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