Literature DB >> 26568242

Intestinal Current Measurements Detect Activation of Mutant CFTR in Patients with Cystic Fibrosis with the G551D Mutation Treated with Ivacaftor.

Simon Y Graeber1,2, Martin J Hug3, Olaf Sommerburg1, Stephanie Hirtz1,2, Julia Hentschel4, Andrea Heinzmann5, Christian Dopfer6, Angela Schulz6,2, Jochen G Mainz4, Burkhard Tümmler6,2, Marcus A Mall1,2.   

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Year:  2015        PMID： 26568242     DOI： 10.1164/rccm.201507-1271LE

Source DB:  PubMed          Journal:  Am J Respir Crit Care Med        ISSN： 1073-449X            Impact factor:   21.405


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  18 in total

1.  Forskolin-induced Swelling in Intestinal Organoids: An In Vitro Assay for Assessing Drug Response in Cystic Fibrosis Patients.

Authors:  Sylvia F Boj; Annelotte M Vonk; Marvin Statia; Jinyi Su; Robert R G Vries; Jeffrey M Beekman; Hans Clevers
Journal:  J Vis Exp       Date:  2017-02-11       Impact factor: 1.355

Review 2.  Personalized medicine in CF: from modulator development to therapy for cystic fibrosis patients with rare CFTR mutations.

Authors:  Misak Harutyunyan; Yunjie Huang; Kyu-Shik Mun; Fanmuyi Yang; Kavisha Arora; Anjaparavanda P Naren
Journal:  Am J Physiol Lung Cell Mol Physiol       Date:  2017-12-14       Impact factor: 5.464

Review 3.  Ion Channel Modulators in Cystic Fibrosis.

Authors:  Martina Gentzsch; Marcus A Mall
Journal:  Chest       Date:  2018-05-08       Impact factor: 9.410

Review 4.  Toward inclusive therapy with CFTR modulators: Progress and challenges.

Authors:  Jennifer Guimbellot; Jyoti Sharma; Steven M Rowe
Journal:  Pediatr Pulmonol       Date:  2017-09-07

Review 5.  CFTR modulator theratyping: Current status, gaps and future directions.

Authors:  John Paul Clancy; Calvin U Cotton; Scott H Donaldson; George M Solomon; Donald R VanDevanter; Michael P Boyle; Martina Gentzsch; Jerry A Nick; Beate Illek; John C Wallenburg; Eric J Sorscher; Margarida D Amaral; Jeffrey M Beekman; Anjaparavanda P Naren; Robert J Bridges; Philip J Thomas; Garry Cutting; Steven Rowe; Anthony G Durmowicz; Martin Mense; Kris D Boeck; William Skach; Christopher Penland; Elizabeth Joseloff; Hermann Bihler; John Mahoney; Drucy Borowitz; Katherine L Tuggle
Journal:  J Cyst Fibros       Date:  2018-06-20       Impact factor: 5.482

Review 6.  Cystic Fibrosis: Emergence of Highly Effective Targeted Therapeutics and Potential Clinical Implications.

Authors:  Marcus A Mall; Nicole Mayer-Hamblett; Steven M Rowe
Journal:  Am J Respir Crit Care Med       Date:  2020-05-15       Impact factor: 21.405

Review 7.  The future of cystic fibrosis care: a global perspective.

Authors:  Scott C Bell; Marcus A Mall; Hector Gutierrez; Milan Macek; Susan Madge; Jane C Davies; Pierre-Régis Burgel; Elizabeth Tullis; Claudio Castaños; Carlo Castellani; Catherine A Byrnes; Fiona Cathcart; Sanjay H Chotirmall; Rebecca Cosgriff; Irmgard Eichler; Isabelle Fajac; Christopher H Goss; Pavel Drevinek; Philip M Farrell; Anna M Gravelle; Trudy Havermans; Nicole Mayer-Hamblett; Nataliya Kashirskaya; Eitan Kerem; Joseph L Mathew; Edward F McKone; Lutz Naehrlich; Samya Z Nasr; Gabriela R Oates; Ciaran O'Neill; Ulrike Pypops; Karen S Raraigh; Steven M Rowe; Kevin W Southern; Sheila Sivam; Anne L Stephenson; Marco Zampoli; Felix Ratjen
Journal:  Lancet Respir Med       Date:  2019-09-27       Impact factor: 30.700

8.  Effect of lumacaftor-ivacaftor on mucociliary clearance and clinical outcomes in cystic fibrosis: Results from the PROSPECT MCC sub-study.

Authors:  Scott H Donaldson; Beth L Laube; Peter Mogayzel; Timothy E Corcoran; Joseph M Pilewski; Agathe Ceppe; Jihong Wu; Pradeep G Bhambhvani; Felix Ratjen; Scott D Sagel; J P Clancy; Steven M Rowe; William D Bennett
Journal:  J Cyst Fibros       Date:  2021-05-31       Impact factor: 5.482

9.  Rescue from Pseudomonas aeruginosa Airway Infection via Stem Cell Transplantation.

Authors:  Kerstin Brinkert; Silke Hedtfeld; Annina Burhop; Rena Gastmeier; Pauline Gad; Dirk Wedekind; Christina Kloth; Justin Rothschuh; Nico Lachmann; Miriam Hetzel; Adan Chari Jirmo; Elena Lopez-Rodriguez; Christina Brandenberger; Gesine Hansen; Axel Schambach; Mania Ackermann; Burkhard Tümmler; Antje Munder
Journal:  Mol Ther       Date:  2020-12-03       Impact factor: 11.454

Review 10.  Recent progress in translational cystic fibrosis research using precision medicine strategies.

Authors:  Deborah M Cholon; Martina Gentzsch
Journal:  J Cyst Fibros       Date:  2017-10-04       Impact factor: 5.482
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