Literature DB >> 32421352

Correlation between Ivacaftor-induced CFTR Activation in Airway Epithelial Cells and Improved Lung Function: A Proof-of-Concept Study.

Jason S Debley1,2, Kaitlyn A Barrow1, Lucille M Rich1, Pradeep Singh2,3, Edward F McKone4,5, David P Nichols1,2.   

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Year:  2020        PMID： 32421352      PMCID： PMC7787006          DOI： 10.1513/AnnalsATS.202001-082RL

Source DB:  PubMed          Journal:  Ann Am Thorac Soc        ISSN： 2325-6621


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  16 in total

1.  Detection of CFTR function and modulation in primary human nasal cell spheroids.

Authors:  John J Brewington; Erin T Filbrandt; F J LaRosa; Alicia J Ostmann; Lauren M Strecker; Rhonda D Szczesniak; John P Clancy
Journal:  J Cyst Fibros       Date:  2017-07-13       Impact factor: 5.482

2.  Change in sweat chloride as a clinical end point in cystic fibrosis clinical trials: the ivacaftor experience.

Authors:  Anthony G Durmowicz; Kimberly A Witzmann; Curtis J Rosebraugh; Badrul A Chowdhury
Journal:  Chest       Date:  2013-01       Impact factor: 9.410

3.  Clinical mechanism of the cystic fibrosis transmembrane conductance regulator potentiator ivacaftor in G551D-mediated cystic fibrosis.

Authors:  Steven M Rowe; Sonya L Heltshe; Tanja Gonska; Scott H Donaldson; Drucy Borowitz; Daniel Gelfond; Scott D Sagel; Umer Khan; Nicole Mayer-Hamblett; Jill M Van Dalfsen; Elizabeth Joseloff; Bonnie W Ramsey
Journal:  Am J Respir Crit Care Med       Date:  2014-07-15       Impact factor: 21.405

4.  A CFTR potentiator in patients with cystic fibrosis and the G551D mutation.

Authors:  Bonnie W Ramsey; Jane Davies; N Gerard McElvaney; Elizabeth Tullis; Scott C Bell; Pavel Dřevínek; Matthias Griese; Edward F McKone; Claire E Wainwright; Michael W Konstan; Richard Moss; Felix Ratjen; Isabelle Sermet-Gaudelus; Steven M Rowe; Qunming Dong; Sally Rodriguez; Karl Yen; Claudia Ordoñez; J Stuart Elborn
Journal:  N Engl J Med       Date:  2011-11-03       Impact factor: 91.245

5.  Correlation of sweat chloride and percent predicted FEV1 in cystic fibrosis patients treated with ivacaftor.

Authors:  Meredith C Fidler; Jack Beusmans; Paul Panorchan; Fredrick Van Goor
Journal:  J Cyst Fibros       Date:  2016-10-20       Impact factor: 5.482

6.  Rectal Organoids Enable Personalized Treatment of Cystic Fibrosis.

Authors:  Gitte Berkers; Peter van Mourik; Annelotte M Vonk; Evelien Kruisselbrink; Johanna F Dekkers; Karin M de Winter-de Groot; Hubertus G M Arets; Rozemarijn E P Marck-van der Wilt; Jasper S Dijkema; Maaike M Vanderschuren; Roderick H J Houwen; Harry G M Heijerman; Eduard A van de Graaf; Sjoerd G Elias; Christof J Majoor; Gerard H Koppelman; Jolt Roukema; Marleen Bakker; Hettie M Janssens; Renske van der Meer; Robert G J Vries; Hans C Clevers; Hugo R de Jonge; Jeffrey M Beekman; Cornelis K van der Ent
Journal:  Cell Rep       Date:  2019-02-12       Impact factor: 9.423

7.  Airway epithelial cells from asthmatic children differentially express proremodeling factors.

Authors:  Jesus M Lopez-Guisa; Claire Powers; Daniele File; Elizabeth Cochrane; Nathalia Jimenez; Jason S Debley
Journal:  J Allergy Clin Immunol       Date:  2012-01-09       Impact factor: 10.793

8.  Treating the Underlying Cystic Fibrosis Transmembrane Conductance Regulator Defect in Patients with Cystic Fibrosis.

Authors:  Senne Cuyx; Kris De Boeck
Journal:  Semin Respir Crit Care Med       Date:  2019-10-28       Impact factor: 3.119

9.  Stability of gene expression by primary bronchial epithelial cells over increasing passage number.

Authors:  Stephen R Reeves; Kaitlyn A Barrow; Maria P White; Lucille M Rich; Maryam Naushab; Jason S Debley
Journal:  BMC Pulm Med       Date:  2018-05-29       Impact factor: 3.317

10.  Deficient Follistatin-like 3 Secretion by Asthmatic Airway Epithelium Impairs Fibroblast Regulation and Fibroblast-to-Myofibroblast Transition.

Authors:  Richard G James; Stephen R Reeves; Kaitlyn A Barrow; Maria P White; Veronika A Glukhova; Candace Haghighi; Dana Seyoum; Jason S Debley
Journal:  Am J Respir Cell Mol Biol       Date:  2018-07       Impact factor: 7.748
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  7 in total

Review 1.  Nasal Epithelial Cell-Based Models for Individualized Study in Cystic Fibrosis.

Authors:  Duncan E Keegan; John J Brewington
Journal:  Int J Mol Sci       Date:  2021-04-24       Impact factor: 5.923

2.  CFTR function and clinical response to modulators parallel nasal epithelial organoid swelling.

Authors:  Justin D Anderson; Zhongyu Liu; L Victoria Odom; Latona Kersh; Jennifer S Guimbellot
Journal:  Am J Physiol Lung Cell Mol Physiol       Date:  2021-05-19       Impact factor: 6.011

Review 3.  Left behind: The potential impact of CFTR modulators on racial and ethnic disparities in cystic fibrosis.

Authors:  Meghan E McGarry; Elizabeth R Gibb; Gabriela R Oates; Michael S Schechter
Journal:  Paediatr Respir Rev       Date:  2021-12-22       Impact factor: 5.526

4.  Avatar acceptability: views from the Australian Cystic Fibrosis community on the use of personalised organoid technology to guide treatment decisions.

Authors:  Laura K Fawcett; Claire E Wakefield; Sheila Sivam; Peter G Middleton; Peter Wark; John Widger; Adam Jaffe; Shafagh A Waters
Journal:  ERJ Open Res       Date:  2021-01-18

5.  Elexacaftor is a CFTR potentiator and acts synergistically with ivacaftor during acute and chronic treatment.

Authors:  Ciaran A Shaughnessy; Pamela L Zeitlin; Preston E Bratcher
Journal:  Sci Rep       Date:  2021-10-06       Impact factor: 4.379

Review 6.  Advances in Preclinical In Vitro Models for the Translation of Precision Medicine for Cystic Fibrosis.

Authors:  Iris A L Silva; Onofrio Laselva; Miquéias Lopes-Pacheco
Journal:  J Pers Med       Date:  2022-08-16

Review 7.  PROMISE: Working with the CF community to understand emerging clinical and research needs for those treated with highly effective CFTR modulator therapy.

Authors:  Dave P Nichols; Scott H Donaldson; Carla A Frederick; Steven D Freedman; Daniel Gelfond; Lucas R Hoffman; Andrea Kelly; Michael R Narkewicz; Jessica E Pittman; Felix Ratjen; Scott D Sagel; Margaret Rosenfeld; Sarah Jane Schwarzenberg; Pradeep K Singh; George M Solomon; Michael S Stalvey; Shannon Kirby; Jill M VanDalfsen; John P Clancy; Steven M Rowe
Journal:  J Cyst Fibros       Date:  2021-02-19       Impact factor: 5.482
  7 in total

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