Literature DB >> 28459323

AJRCCM: 100-Year Anniversary. Progress along the Pathway of Discovery Leading to Treatment and Cure of Cystic Fibrosis.

Bonnie W Ramsey1,2, Michael J Welsh3,4,5.   

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Year:  2017        PMID： 28459323      PMCID： PMC5439020          DOI： 10.1164/rccm.201702-0266ED

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


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  71 in total

1.  Future directions in early cystic fibrosis lung disease research: an NHLBI workshop report.

Authors:  Bonnie W Ramsey; Susan Banks-Schlegel; Frank J Accurso; Richard C Boucher; Garry R Cutting; John F Engelhardt; William B Guggino; Christopher L Karp; Michael R Knowles; Jay K Kolls; John J LiPuma; Susan Lynch; Paul B McCray; Ronald C Rubenstein; Pradeep K Singh; Eric Sorscher; Michael Welsh
Journal:  Am J Respir Crit Care Med       Date:  2012-02-03       Impact factor: 21.405

2.  Discovery of Clinically Approved Agents That Promote Suppression of Cystic Fibrosis Transmembrane Conductance Regulator Nonsense Mutations.

Authors:  Venkateshwar Mutyam; Ming Du; Xiaojiao Xue; Kim M Keeling; E Lucile White; J Robert Bostwick; Lynn Rasmussen; Bo Liu; Marina Mazur; Jeong S Hong; Emily Falk Libby; Feng Liang; Haibo Shang; Martin Mense; Mark J Suto; David M Bedwell; Steven M Rowe
Journal:  Am J Respir Crit Care Med       Date:  2016-11-01       Impact factor: 21.405

Review 3.  Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease.

Authors:  A S Verkman; Yuanlin Song; Jay R Thiagarajah
Journal:  Am J Physiol Cell Physiol       Date:  2003-01       Impact factor: 4.249

Review 4.  Cystic fibrosis genetics: from molecular understanding to clinical application.

Authors:  Garry R Cutting
Journal:  Nat Rev Genet       Date:  2014-11-18       Impact factor: 53.242

5.  Direct sampling of cystic fibrosis lungs indicates that DNA-based analyses of upper-airway specimens can misrepresent lung microbiota.

Authors:  Amanda F Goddard; Benjamin J Staudinger; Scot E Dowd; Amruta Joshi-Datar; Randall D Wolcott; Moira L Aitken; Corinne L Fligner; Pradeep K Singh
Journal:  Proc Natl Acad Sci U S A       Date:  2012-08-07       Impact factor: 11.205

6.  Chloride impermeability in cystic fibrosis.

Authors:  P M Quinton
Journal:  Nature       Date:  1983-02-03       Impact factor: 49.962

7.  Lung disease at diagnosis in infants with cystic fibrosis detected by newborn screening.

Authors:  Peter D Sly; Siobhain Brennan; Catherine Gangell; Nicholas de Klerk; Conor Murray; Lauren Mott; Stephen M Stick; Philip J Robinson; Colin F Robertson; Sarath C Ranganathan
Journal:  Am J Respir Crit Care Med       Date:  2009-04-16       Impact factor: 21.405

8.  Identification of the cystic fibrosis gene: chromosome walking and jumping.

Authors:  J M Rommens; M C Iannuzzi; B Kerem; M L Drumm; G Melmer; M Dean; R Rozmahel; J L Cole; D Kennedy; N Hidaka
Journal:  Science       Date:  1989-09-08       Impact factor: 47.728

9.  Ataluren for the treatment of nonsense-mutation cystic fibrosis: a randomised, double-blind, placebo-controlled phase 3 trial.

Authors:  Eitan Kerem; Michael W Konstan; Kris De Boeck; Frank J Accurso; Isabelle Sermet-Gaudelus; Michael Wilschanski; J Stuart Elborn; Paola Melotti; Inez Bronsveld; Isabelle Fajac; Anne Malfroot; Daniel B Rosenbluth; Patricia A Walker; Susanna A McColley; Christiane Knoop; Serena Quattrucci; Ernst Rietschel; Pamela L Zeitlin; Jay Barth; Gary L Elfring; Ellen M Welch; Arthur Branstrom; Robert J Spiegel; Stuart W Peltz; Temitayo Ajayi; Steven M Rowe
Journal:  Lancet Respir Med       Date:  2014-05-15       Impact factor: 30.700

10.  Characterization of defects in ion transport and tissue development in cystic fibrosis transmembrane conductance regulator (CFTR)-knockout rats.

Authors:  Katherine L Tuggle; Susan E Birket; Xiaoxia Cui; Jeong Hong; Joe Warren; Lara Reid; Andre Chambers; Diana Ji; Kevin Gamber; Kengyeh K Chu; Guillermo Tearney; Li Ping Tang; James A Fortenberry; Ming Du; Joan M Cadillac; David M Bedwell; Steven M Rowe; Eric J Sorscher; Michelle V Fanucchi
Journal:  PLoS One       Date:  2014-03-07       Impact factor: 3.240
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  7 in total

1.  The Pathophysiology of Rett Syndrome With a Focus on Breathing Dysfunctions.

Authors:  Jan-Marino Ramirez; Marlusa Karlen-Amarante; Jia-Der Ju Wang; Nicholas E Bush; Michael S Carroll; Debra E Weese-Mayer; Alyssa Huff
Journal:  Physiology (Bethesda)       Date:  2020-11-01

2.  On the Process of Discovering Leads That Target the Heparin-Binding Site of Neutrophil Elastase in the Sputum of Cystic Fibrosis Patients.

Authors:  Shravan Morla; Nehru Viji Sankaranarayanan; Daniel K Afosah; Megh Kumar; Apparao B Kummarapurugu; Judith A Voynow; Umesh R Desai
Journal:  J Med Chem       Date:  2019-05-28       Impact factor: 7.446

3.  Molecular principles for heparin oligosaccharide-based inhibition of neutrophil elastase in cystic fibrosis.

Authors:  Apparao B Kummarapurugu; Daniel K Afosah; Nehru Viji Sankaranarayanan; Rahaman Navaz Gangji; Shuo Zheng; Thomas Kennedy; Bruce K Rubin; Judith A Voynow; Umesh R Desai
Journal:  J Biol Chem       Date:  2018-06-14       Impact factor: 5.157

4.  CNS imaging studies in cystic fibrosis patients presenting with sudden neurological events.

Authors:  Samantha Ellis; Catherine Rang; Tom Kotsimbos; Dominic Keating; Felicity Finlayson; Richard Stark; Dominic Thyagarajan; John Wilson
Journal:  BMJ Open Respir Res       Date:  2019-08-09

5.  A small molecule CFTR potentiator restores ATP-dependent channel gating to the cystic fibrosis mutant G551D-CFTR.

Authors:  Jia Liu; Allison P Berg; Yiting Wang; Walailak Jantarajit; Katy J Sutcliffe; Edward B Stevens; Lishuang Cao; Marko J Pregel; David N Sheppard
Journal:  Br J Pharmacol       Date:  2022-01-21       Impact factor: 9.473

6.  CFTR Modulator Therapy with Lumacaftor/Ivacaftor Alters Plasma Concentrations of Lipid-Soluble Vitamins A and E in Patients with Cystic Fibrosis.

Authors:  Olaf Sommerburg; Susanne Hämmerling; S Philipp Schneider; Jürgen Okun; Claus-Dieter Langhans; Patricia Leutz-Schmidt; Mark O Wielpütz; Werner Siems; Simon Y Gräber; Marcus A Mall; Mirjam Stahl
Journal:  Antioxidants (Basel)       Date:  2021-03-19

7.  Journey on VX-809-Based Hybrid Derivatives towards Drug-like F508del-CFTR Correctors: From Molecular Modeling to Chemical Synthesis and Biological Assays.

Authors:  Alice Parodi; Giada Righetti; Emanuela Pesce; Annalisa Salis; Valeria Tomati; Cristina Pastorino; Bruno Tasso; Mirko Benvenuti; Gianluca Damonte; Nicoletta Pedemonte; Elena Cichero; Enrico Millo
Journal:  Pharmaceuticals (Basel)       Date:  2022-02-23
  7 in total

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