Keyword

CFTR

Description: CFTR is a human gene encoding a membrane protein that transports chloride and other ions and is associated with cystic fibrosis.

Primary topic: Cystic Fibrosis Research Advances (62%)

Wikidata ID: wiki/Q14864712

OpenAlex ID: keywords/cftr

Update on Key Emerging Challenges in Cystic Fibrosis

2014 · Mark T. Jennings, Kristin A. Riekert, et al. · Medical Principles and Practice · 3,648

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Aggresomes: A Cellular Response to Misfolded Proteins

1998 · Jennifer A. Johnston, Cristina L. Ward, et al. · The Journal of Cell Biology · 2,147

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Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

1990 · Seng H. Cheng, Richard J. Gregory, et al. · Cell · 1,792

Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR

2015 · Claire E. Wainwright, J. Stuart Elborn, et al. · New England Journal of Medicine · 1,631

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Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis

1993 · Michael J. Welsh, Alan E. Smith · Cell · 1,490

Functional Repair of CFTR by CRISPR/Cas9 in Intestinal Stem Cell Organoids of Cystic Fibrosis Patients

2013 · Gerald Schwank, Bon-Kyoung Koo, et al. · Cell stem cell · 1,423

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Cystic fibrosis

2009 · Brian P O'Sullivan, Steven D Freedman · The Lancet · 1,319

Degradation of CFTR by the ubiquitin-proteasome pathway

1995 · Cristina L. Ward, Satoshi Omura, et al. · Cell · 1,304

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Molecular Structure and Physiological Function of Chloride Channels

2002 · Thomas J. Jentsch, Valentin Stein, et al. · Physiological Reviews · 1,257

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

1992 · Gerene M. Denning, Matthew P. Anderson, et al. · Nature · 1,234

Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

2009 · Fredrick Van Goor, Sabine Hadida, et al. · Proceedings of the National Academy of Sciences · 1,228

A revised airway epithelial hierarchy includes CFTR-expressing ionocytes

2018 · Daniel T. Montoro, Adam L. Haber, et al. · Nature · 1,204

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Evidence for Periciliary Liquid Layer Depletion, Not Abnormal Ion Composition, in the Pathogenesis of Cystic Fibrosis Airways Disease

1998 · Hirotoshi Matsui, Barbara R Grubb, et al. · Cell · 1,169

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Genomic Landscape of Non-Small Cell Lung Cancer in Smokers and Never-Smokers

2012 · Ramaswamy Govindan, Li Ding, et al. · Cell · 1,147

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Structural model of ATP-binding proteing associated with cystic fibrosis, multidrug resistance and bacterial transport

1990 · Stephen C. Hyde, Paul Emsley, et al. · Nature · 1,145

Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity

1991 · Matthew P. Anderson, Richard J. Gregory, et al. · Science · 1,138

CFTR as a cAMP-Dependent Regulator of Sodium Channels

1995 · M. Jackson Stutts, Cecilia M. Canessa, et al. · Science · 1,088

A single-cell atlas of the airway epithelium reveals the CFTR-rich pulmonary ionocyte

2018 · Lindsey W. Plasschaert, Rapolas Žilionis, et al. · Nature · 1,048

The future of cystic fibrosis care: a global perspective

2019 · Scott C Bell, Marcus A Mall, et al. · The Lancet Respiratory Medicine · 1,037

Structure and Function of the CFTR Chloride Channel

1999 · DAVID N. SHEPPARD, MICHAEL J. WELSH · Physiological Reviews · 1,018

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A functional CFTR assay using primary cystic fibrosis intestinal organoids

2013 · Johanna F Dekkers, Caroline L Wiegerinck, et al. · Nature Medicine · 1,000

Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)

1992 · Christine E. Bear, Canhui Li, et al. · Cell · 945

CFTR Expression and Chloride Secretion in Polarized Immortal Human Bronchial Epithelial Cells

1994 · A L Cozens, M J Yezzi, et al. · American Journal of Respiratory Cell and Molecular Biology · 929

An Animal Model for Cystic Fibrosis Made by Gene Targeting

1992 · John N. Snouwaert, Kristen K. Brigman, et al. · Science · 918

Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

2004 · Marcus Mall, Barbara R Grubb, et al. · Nature Medicine · 911

Works count: 16,030

Citation count: 424,300