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Most cited papers in Cystic Fibrosis Research Advances

What are the most cited papers in Cystic Fibrosis Research Advances?

The most cited paper in Cystic Fibrosis Research Advances is "Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA" (John R. Riordan, Johanna M. Rommens, Bat-Sheva Kerem et al., 1989), with 7,480 citations. Next come "Modulating Innate and Adaptive Immunity by (R)-Roscovitine: Potential Therapeutic Opportunity in Cystic Fibrosis" (4,999) and "Standardization of Spirometry 2019 Update. An Official American Thoracic Society and European Respiratory Society Technical Statement" (4,326).

Showing 50 of 99 papers. Every paper with more than 831 citations is listed.

Most cited papers, sortable by citations and release date
#Paper
1 Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNA John R. Riordan, Johanna M. Rommens, Bat-Sheva Kerem et al. · Science 8 Sep 1989 7,480
2 Modulating Innate and Adaptive Immunity by (R)-Roscovitine: Potential Therapeutic Opportunity in Cystic Fibrosis Laurent Meijer et al. · Journal of Innate Immunity Open access 2016 4,999
3 Standardization of Spirometry 2019 Update. An Official American Thoracic Society and European Respiratory Society Technical Statement Brian L. Graham, Irene Steenbruggen, Martin R. Miller et al. · American Journal of Respiratory and Critical Care Medicine Open access Oct 2019 4,326
4 Identification of the Cystic Fibrosis Gene: Genetic Analysis Bat-Sheva Kerem, Johanna M. Rommens, Janet A. Buchanan et al. · Science 8 Sep 1989 4,164
5 Combination of 16S rRNA-targeted oligonucleotide probes with flow cytometry for analyzing mixed microbial populations Rudolf Amann, Brian J. Binder, Robert Olson et al. · Applied and Environmental Microbiology Open access Jun 1990 4,069
6 Update on Key Emerging Challenges in Cystic Fibrosis Mark T. Jennings, Kristin A. Riekert, Michael Boyle · Medical Principles and Practice Open access 2014 3,663
7 A Comparison of Bayesian Methods for Haplotype Reconstruction from Population Genotype Data Matthew Stephens, Peter Donnelly · The American Journal of Human Genetics Open access 24 Oct 2003 3,550
8 Identification of the Cystic Fibrosis Gene: Chromosome Walking and Jumping Johanna M. Rommens, Michael C. Iannuzzi, Bat-Sheva Kerem et al. · Science 8 Sep 1989 3,269
9 Clinical Practice Guidelines for the Management Candidiasis: 2009 Update by the Infectious Diseases Society of America Peter G. Pappas, Carol A. Kauffman, David R. Andes et al. · Clinical Infectious Diseases Open access 29 Jan 2009 3,216
10 Therapy of CF-Patients with Amitriptyline and Placebo - a Randomised, Double-Blind, Placebo-Controlled Phase IIb Multicenter, Cohort-Study Lutz Nährlich, Jochen Georg Mainz, Constantin Adams et al. · Cellular Physiology and Biochemistry Open access 2013 2,535
11 Characterization of the Human Colon Carcinoma Cell Line (Caco-2) as a Model System for Intestinal Epithelial Permeability Ismael J. Hidalgo, Thomas J. Raub, Ronald T. Borchardt · Gastroenterology Feb 1989 2,226
12 A CFTR Potentiator in Patients with Cystic Fibrosis and the G551D Mutation Bonnie W. Ramsey, Jane Carolyn Davies, Noel Gerard McElvaney et al. · New England Journal of Medicine Open access 2 Nov 2011 2,213
13 Elexacaftor–Tezacaftor–Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele Peter Gordon Middleton et al. · New England Journal of Medicine Open access 31 Oct 2019 2,211
14 Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials Paul W. Noble, Carlo Albera, Williamson Z. Bradford et al. · The Lancet May 2011 2,182
15 Aggresomes: A Cellular Response to Misfolded Proteins Jennifer A. Johnston, Cristina L. Ward, Ron R. Kopito · The Journal of Cell Biology Open access 28 Dec 1998 2,172
16 Estimating cumulative point prevalence of rare diseases: analysis of the Orphanet database Stéphanie Nguengang Wakap, Deborah M. Lambert, Annie Olry et al. · European Journal of Human Genetics Open access 16 Sep 2019 1,868
17 Cystic fibrosis J.S. Elborn · The Lancet May 2016 1,718
18 Review on uremic toxins: Classification, concentration, and interindividual variability Raymond Camille Vanholder et al. · Kidney International Open access May 2003 1,709
19 Microbial pathogenesis in cystic fibrosis: mucoid Pseudomonas aeruginosa and Burkholderia cepacia John R. W. Govan, Vojo P. Deretic · Microbiological Reviews Open access Sep 1996 1,672
20 Lung Infections Associated with Cystic Fibrosis Jeffrey B. Lyczak, Carolyn L. Cannon, Gerald B. Pier · Clinical Microbiology Reviews Open access Apr 2002 1,656
21 Respiratory syncytial virus in early life and risk of wheeze and allergy by age 13 years Renato Tetelbom Stein, Duane L. Sherrill, Wayne J. Morgan et al. · The Lancet Aug 1999 1,650
22 Lumacaftor–Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR Claire Elizabeth Wainwright, J.S. Elborn, Bonnie W. Ramsey et al. · New England Journal of Medicine Open access 17 May 2015 1,650
23 ERS/ATS technical standard on interpretive strategies for routine lung function tests Sanja Stanojevic, David A. Kaminsky, Martin R. Miller et al. · European Respiratory Journal Open access 23 Dec 2021 1,639
24 Pathophysiology and Management of Pulmonary Infections in Cystic Fibrosis Ronald L. Gibson, Jane L. Burns, Bonnie W. Ramsey · American Journal of Respiratory and Critical Care Medicine Oct 2003 1,639
25 Effect of Aerosolized Recombinant Human DNase on Exacerbations of Respiratory Symptoms and on Pulmonary Function in Patients with Cystic Fibrosis Henry J. Fuchs, Drucy Borowitz, David Høyrup Christiansen et al. · New England Journal of Medicine Open access 8 Sep 1994 1,543
26 Quorum-sensing in Gram-negative bacteria Neil A. Whitehead, Anne M. L. Barnard, Holly Slater et al. · FEMS Microbiology Reviews Aug 2001 1,527
27 Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis Michael J. Welsh, Alan Edward Smith · Cell Jul 1993 1,491
28 High Frequency of Hypermutable Pseudomonas aeruginosa in Cystic Fibrosis Lung Infection Antonio Oliver, Rafael Cantón, Pilar Campo et al. · Science 19 May 2000 1,489
29 A Longitudinal, Population-Based, Cohort Study of Childhood Asthma Followed to Adulthood Malcolm R. Sears, Justina M. Greene, Andrew R. Willan et al. · New England Journal of Medicine Open access 8 Oct 2003 1,472
30 A TEST FOR CONCENTRATION OF ELECTROLYTES IN SWEAT IN CYSTIC FIBROSIS OF THE PANCREAS UTILIZING PILOCARPINE BY IONTOPHORESIS Lewis E. Gibson, Robert E. Cooke · PEDIATRICS Mar 1959 1,439
31 Functional Repair of CFTR by CRISPR/Cas9 in Intestinal Stem Cell Organoids of Cystic Fibrosis Patients Gerald Schwank, Bon‐Kyoung Koo, Valentina Sasselli et al. · Cell stem cell Open access Dec 2013 1,429
32 Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial Harry G.M. Heijerman et al. · The Lancet Open access Nov 2019 1,420
33 Cystic Fibrosis Steven Mark Rowe, Stacey Miller, Eric J. Sorscher · New England Journal of Medicine 11 May 2005 1,413
34 Bronchiolitis-Associated Hospitalizations Among US Children, 1980-1996 David K. Shay · JAMA 20 Oct 1999 1,395
35 European Respiratory Society guidelines for the management of adult bronchiectasis Eva Polverino et al. · European Respiratory Journal Open access Sep 2017 1,387
36 The hierarchy quorum sensing network in Pseudomonas aeruginosa Jasmine R. Lee, Lian‐Hui Zhang · Protein & Cell Open access 23 Sep 2014 1,380
37 Genetic adaptation by Pseudomonas aeruginosa to the airways of cystic fibrosis patients Eric E. Smith, Danielle Buckley, Zaining Wu et al. · Proceedings of the National Academy of Sciences 11 May 2006 1,369
38 Degradation of CFTR by the ubiquitin-proteasome pathway Cristina L. Ward, Satoshi ŌMURA, Ron R. Kopito · Cell Open access Oct 1995 1,320
39 Cystic fibrosis Brian P O'Sullivan, Steven D. Freedman · The Lancet May 2009 1,318
40 Intermittent Administration of Inhaled Tobramycin in Patients with Cystic Fibrosis Bonnie W. Ramsey, MARGARET SULLIVAN PEPE, Joanne M. Quan et al. · New England Journal of Medicine Open access 7 Jan 1999 1,309
41 The Measurement of Dyspnea Donald A. Mahler, David H. Weinberg, Carolyn K. Wells et al. · CHEST Journal Jun 1984 1,303
42 Continuous Fluorescence Monitoring of Rapid Cycle DNA Amplification Carl T. Wittwer, Mark G. Herrmann, Alan A. Moss et al. · BioTechniques Open access Jun 2013 1,280
43 Protein Degradation by the Ubiquitin–Proteasome Pathway in Normal and Disease States Stewart H. Lecker, Alfred Lewis Goldberg, William E. Mitch · Journal of the American Society of Nephrology 31 May 2006 1,263
44 Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive Gerene M. Denning, Matthew P. Anderson, Jane F. Amara et al. · Nature Aug 1992 1,248
45 Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770 Fredrick Van Goor et al. · Proceedings of the National Academy of Sciences Open access 21 Oct 2009 1,245
46 Human Inborn Errors of Immunity: 2019 Update on the Classification from the International Union of Immunological Societies Expert Committee Stuart G. Tangye, Waleed Al–Herz, Aziz A. Bousfiha et al. · Journal of Clinical Immunology Open access 2020 1,238
47 Early Pulmonary Inflammation in Infants with Cystic Fibrosis Talat Z. Khan, Jeffrey S. Wagener, Thomas Bost et al. · American Journal of Respiratory and Critical Care Medicine Apr 1995 1,211
48 Characterisation of COPD heterogeneity in the ECLIPSE cohort the Evaluation of COPD Longitudinally to Identify Predictive Surrogate Endpoints, Alvar G.N. Agustí, Peter M.A. Calverley et al. · Respiratory Research Open access 10 Sep 2010 1,169
49 Evidence for Periciliary Liquid Layer Depletion, Not Abnormal Ion Composition, in the Pathogenesis of Cystic Fibrosis Airways Disease Hirotoshi Matsui, Barbara R. Grubb, Robert Tarran et al. · Cell Open access Dec 1998 1,167
50 Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity Matthew P. Anderson, Richard J. Gregory, Simon John Thompson et al. · Science 12 Jul 1991 1,144

Citations are OpenAlex's own counts, from its 2026-09-23 snapshot, so the ranking can differ from Google Scholar or Web of Science. A paper is listed for a field when OpenAlex files it under one of the field's topics; retracted papers and papers whose citation count cannot be traced to citing works are left out, and a preprint and the article it became are shown once. Release dates are OpenAlex's: where a source gave only a month or a year, that is what is shown. How the lists are chosen.