Literature DB >> 26071414

Lumacaftor/ivacaftor for patients homozygous for Phe508del-CFTR: should we curb our enthusiasm?

Andrew M Jones1, Peter J Barry1.   

Abstract

Entities:  

Keywords:  Cystic Fibrosis

Mesh：

Substances：

Year:  2015        PMID： 26071414     DOI： 10.1136/thoraxjnl-2015-207369

Source DB:  PubMed          Journal:  Thorax        ISSN： 0040-6376            Impact factor:   9.139


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  15 in total

Review 1.  Lumacaftor/Ivacaftor: A Review in Cystic Fibrosis.

Authors:  Emma D Deeks
Journal:  Drugs       Date:  2016-08       Impact factor: 9.546

2.  Nasospheroids permit measurements of CFTR-dependent fluid transport.

Authors:  Jennifer S Guimbellot; Justin M Leach; Imron G Chaudhry; Nancy L Quinney; Susan E Boyles; Michael Chua; Inmaculada Aban; Ilona Jaspers; Martina Gentzsch
Journal:  JCI Insight       Date:  2017-11-16

Review 3.  Can Cystic Fibrosis Patients Finally Catch a Breath With Lumacaftor/Ivacaftor?

Authors:  E K Schneider; F Reyes-Ortega; J Li; T Velkov
Journal:  Clin Pharmacol Ther       Date:  2016-11-23       Impact factor: 6.875

Review 4.  Barriers to inhaled gene therapy of obstructive lung diseases: A review.

Authors:  Namho Kim; Gregg A Duncan; Justin Hanes; Jung Soo Suk
Journal:  J Control Release       Date:  2016-05-16       Impact factor: 9.776

5.  The expression of Mirc1/Mir17-92 cluster in sputum samples correlates with pulmonary exacerbations in cystic fibrosis patients.

Authors:  Kathrin Krause; Benjamin T Kopp; Mia F Tazi; Kyle Caution; Kaitlin Hamilton; Asmaa Badr; Chandra Shrestha; Dmitry Tumin; Don Hayes; Frank Robledo-Avila; Luanne Hall-Stoodley; Brett G Klamer; Xiaoli Zhang; Santiago Partida-Sanchez; Narasimham L Parinandi; Stephen E Kirkby; Duaa Dakhlallah; Karen S McCoy; Estelle Cormet-Boyaka; Amal O Amer
Journal:  J Cyst Fibros       Date:  2017-12-11       Impact factor: 5.482

6.  Potentiators exert distinct effects on human, murine, and Xenopus CFTR.

Authors:  Guiying Cui; Netaly Khazanov; Brandon B Stauffer; Daniel T Infield; Barry R Imhoff; Hanoch Senderowitz; Nael A McCarty
Journal:  Am J Physiol Lung Cell Mol Physiol       Date:  2016-06-10       Impact factor: 5.464

7.  Connectivity mapping (ssCMap) to predict A20-inducing drugs and their antiinflammatory action in cystic fibrosis.

Authors:  Beth Malcomson; Hollie Wilson; Eleonora Veglia; Gayathri Thillaiyampalam; Ruth Barsden; Shauna Donegan; Amal El Banna; Joseph S Elborn; Madeleine Ennis; Catriona Kelly; Shu-Dong Zhang; Bettina C Schock
Journal:  Proc Natl Acad Sci U S A       Date:  2016-06-10       Impact factor: 11.205

Review 8.  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

9.  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 10.  Manipulating proteostasis to repair the F508del-CFTR defect in cystic fibrosis.

Authors:  Speranza Esposito; Antonella Tosco; Valeria R Villella; Valeria Raia; Guido Kroemer; Luigi Maiuri
Journal:  Mol Cell Pediatr       Date:  2016-03-14
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