ArticleNature medicine2021
Transcriptional analysis of cystic fibrosis airways at single-cell resolution reveals altered epithelial cell states and composition.
Open access · greenAbstract readMulticenter Study
In one paragraph
Article in Nature medicine, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 115 papers.
0numbers the graph read from it
0cells of the map it votes in
115citing papers in PubMed
21.4field-weighted citation impact, top 1% of its field
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
115 citing papers in PubMed, 176 citations in OpenAlex.
- Alveolar Macrophages: Development, Respiratory Homeostasis, Signal Pathways, Diseases, and Therapeutic Opportunities.MedComm · 2026Review
- Developmental dynamics of ovine lung in health and cystic fibrosis at single-cell resolution.Functional & integrative genomics · 2026Article
- Cx43 levels guide apicobasal polarity in regenerating airway epithelial cells.Communications biology · 2026Article
- Base editing and nanoparticle transfection of airway cell types essential for treatment of cystic fibrosis.JCI insight · 2026Article
- Genetic and chemical correction of cystic fibrosis reduces airway susceptibility to SARS-CoV-2.American journal of physiology. Lung cellular and molecular physiology · 2026Article
- The single-cell transcriptional landscape of the pediatric cystic fibrosis lung from minimally invasive respiratory specimens.Scientific reports · 2026Article
- Airway epithelial heterogeneity and mucus plugging in asthmatic bronchioles.American journal of respiratory and critical care medicine · 2026Article
- Steering the course: targeting and exploiting surface receptors in phage therapy.Journal of bacteriology · 2026Review
- Distinct regulatory elements of SLC6A14 expression contribute to modification of cystic fibrosis phenotypes.Human genetics · 2026Article
- Extracellular vesicle-based delivery to airway basal cells for durable gene therapy in cystic fibrosis.Frontiers in bioengineering and biotechnology · 2026Review
- Single cell and spatial characterization of the human pancreas reveals drivers of beta cell dysfunction in cystic fibrosis.bioRxiv : the preprint server for biology · 2025Article
- Single-cell RNA-sequencing co-expression analysis with CFTR and CF modifier genes in lung tissue.Scientific reports · 2025Article
- Comparative Single-Cell Transcriptomics Uncovers Shared and Distinct Molecular Signatures in Cystic Fibrosis and Primary Ciliary Dyskinesia.bioRxiv : the preprint server for biology · 2025Article
- Transcriptomic and functional responses of the cystic fibrosis airway epithelium to CFTR modulator therapy.JCI insight · 2025Article
- Base editing and nanoparticle transfection of airway cell types essential for treatment of cystic fibrosis.bioRxiv : the preprint server for biology · 2025Article
- Characteristics of ectopic alveolar basal cells relative to airway basal cells in fibrosis.ERJ open research · 2025Article
- Cystic fibrosis airway epithelial cells have an exaggerated response to hypoxia that is partially reversible through transmembrane receptor modulation.Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025Article
- Human bronchial basal cells are a community of variants.iScience · 2025Article
- STAT3-Dependent Regulation of CFTR and Ciliogenesis Is Essential for Mucociliary Clearance and Innate Airway Defense in Hyper-IgE Syndrome.American journal of respiratory and critical care medicine · 2025Article
- Pulmonary Ionocytes at the Crossroads of Absorption and Secretion: Clues from the Ferret.American journal of respiratory and critical care medicine · 2025Article
55 more citing papers are in PubMed but not listed here.
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
29 authors at 6 institutions in 1 country.
Gianni Carraro *Lung and Regenerative Medicine Institutes, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.ORCID http://orcid.org/0000-0003-0824-7692
Justin Langerman *Department of Biological Chemistry, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Shan SabriDepartment of Biological Chemistry, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Zareeb LorenzanaHastings Center for Pulmonary Research and Division of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
Arunima PurkayasthaUCLA Children's Discovery and Innovation Institute, Mattel Children's Hospital UCLA, Department of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Guangzhu ZhangLung and Regenerative Medicine Institutes, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Bindu KondaLung and Regenerative Medicine Institutes, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Cody J ArosUCLA Children's Discovery and Innovation Institute, Mattel Children's Hospital UCLA, Department of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Ben A CalvertHastings Center for Pulmonary Research and Division of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
Aleks SzymaniakCFFT Lab, Cystic Fibrosis Foundation, Lexington, MA, USA.
Emily WilsonCFFT Lab, Cystic Fibrosis Foundation, Lexington, MA, USA.ORCID http://orcid.org/0000-0002-0397-6514
Michael MulliganCFFT Lab, Cystic Fibrosis Foundation, Lexington, MA, USA.
Priyanka BhattCFFT Lab, Cystic Fibrosis Foundation, Lexington, MA, USA.ORCID http://orcid.org/0000-0002-4531-6952
Junjie LuCFFT Lab, Cystic Fibrosis Foundation, Lexington, MA, USA.ORCID http://orcid.org/0000-0002-4175-1762
Preethi VijayarajUCLA Children's Discovery and Innovation Institute, Mattel Children's Hospital UCLA, Department of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Changfu YaoLung and Regenerative Medicine Institutes, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
David W ShiaUCLA Children's Discovery and Innovation Institute, Mattel Children's Hospital UCLA, Department of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Andrew J LundUCLA Children's Discovery and Innovation Institute, Mattel Children's Hospital UCLA, Department of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Edo IsraelyLung and Regenerative Medicine Institutes, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
Tammy M RickabaughUCLA Children's Discovery and Innovation Institute, Mattel Children's Hospital UCLA, Department of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Jason ErnstDepartment of Biological Chemistry, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.ORCID http://orcid.org/0000-0003-4026-7853
Martin MenseCFFT Lab, Cystic Fibrosis Foundation, Lexington, MA, USA.ORCID http://orcid.org/0000-0003-0280-9789
Scott H RandellMarsico Lung Institute/Cystic Fibrosis Center, University of North Carolina, Chapel Hill, NC, USA.ORCID http://orcid.org/0000-0001-5351-2841
Eszter K VladarDivision of Pulmonary Sciences and Critical Care Medicine, Department of Medicine and Department of Cell and Developmental Biology, University of Colorado Denver School of Medicine, Aurora, CO, USA.ORCID http://orcid.org/0000-0002-4160-8894
Amy L RyanHastings Center for Pulmonary Research and Division of Pulmonary, Critical Care and Sleep Medicine, Department of Medicine, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
Kathrin PlathDepartment of Biological Chemistry, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA. kplath@mednet.ucla.edu.ORCID http://orcid.org/0000-0001-7796-3372
John E MahoneyCFFT Lab, Cystic Fibrosis Foundation, Lexington, MA, USA. jmahoney@cff.org.ORCID http://orcid.org/0000-0001-9414-9110
Barry R StrippLung and Regenerative Medicine Institutes, Department of Medicine, Cedars-Sinai Medical Center, Los Angeles, CA, USA. barry.stripp@cshs.org.ORCID http://orcid.org/0000-0003-4348-2137
Brigitte N GompertsUCLA Children's Discovery and Innovation Institute, Mattel Children's Hospital UCLA, Department of Pediatrics, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA. bgomperts@mednet.ucla.edu.ORCID http://orcid.org/0000-0002-1809-723X
University of California, Los Angeles · USCystic Fibrosis Foundation · USCedars-Sinai Medical Center · USUniversity of Southern California · USUniversity of Colorado Denver · USUniversity of North Carolina at Chapel Hill · US
Funding
UCLA-Caltech Medical Scientist Training ProgramT32GM008042 · NIGMS · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI AJIJOLA, OLUJIMI A, DAWSON, DAVID WAYNE · 1985 to 2023
$29.9MVector CoreP30DK065988 · NIDDK · UNIV OF NORTH CAROLINA CHAPEL HILL · PI Scott H Randell · 2004 to 2026
$26.5MRole of Surfactant Proteins in Mediating Noninfectious Inflammatory and FibroproP01HL108793 · NHLBI · DUKE UNIVERSITY · PI NOBLE, PAUL WESLEY · 2012 to 2022
$21.3MRoles of sumoylation and alternative polyadenylation in cellular reprogrammingP01GM099134 · NIGMS · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI HOCHEDLINGER, KONRAD · 2011 to 2021
$20.3MThe Effect of Nanoparticle Encapsulated Resveratrol on Treatment of PeriodontitisKL2TR001882 · NCATS · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI Elizabeta Nemeth · 2016 to 2026
$13.1MTUMOR CELL SURFACES AND CHROMOSOMEST32CA009056 · NCI · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI Gay M Crooks, David A. Nathanson · 1985 to 2026
$10.5MDeciphering the Relationship between Substance Use and Psychiatric Disorders from Whole Genome Sequencing DataDP1DA044371 · NIDA · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI ERNST, JASON · 2017 to 2021
$2.3MDeveloping a targeted chemoprevention strategy for Non-Small Cell Lung CancerR01CA208303 · NCI · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI GOMPERTS, BRIGITTE N · 2016 to 2020
$1.9MEvaluating the Role and Regulation of Wnt Signaling in Proximal Airway Homeostasis and Injury RepairF31CA239655 · NCI · UNIVERSITY OF CALIFORNIA LOS ANGELES · PI AROS, CODY JAMES · 2019 to 2020
$79kHoward Hughes Medical InstituteNCATS NIH HHS KL2 TR001882NCI NIH HHS F31 CA239655NCI NIH HHS R01 CA208303NCI NIH HHS T32 CA009056NCIRD CDC HHS U66 IP000597NHLBI NIH HHS P01 HL108793NIDA NIH HHS DP1 DA044371NIDDK NIH HHS P30 DK065988NIGMS NIH HHS P01 GM099134NIGMS NIH HHS T32 GM008042
Abstract
Cystic fibrosis (CF) is a lethal autosomal recessive disorder that afflicts more than 70,000 people. People with CF experience multi-organ dysfunction resulting from aberrant electrolyte transport across polarized epithelia due to mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. CF-related lung disease is by far the most important determinant of morbidity and mortality. Here we report results from a multi-institute consortium in which single-cell transcriptomics were applied to define disease-related changes by comparing the proximal airway of CF donors (n = 19) undergoing transplantation for end-stage lung disease with that of previously healthy lung donors (n = 19). Disease-dependent differences observed include an overabundance of epithelial cells transitioning to specialized ciliated and secretory cell subsets coupled with an unexpected decrease in cycling basal cells. Our study yields a molecular atlas of the proximal airway epithelium that will provide insights for the development of new targeted therapies for CF airway disease.
Indexed as
Cell DifferentiationCiliaCystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorEpithelial CellsGene Expression ProfilingHumansLungRespiratory MucosaSingle-Cell AnalysisTranscriptomeCFTR protein, humanCystic Fibrosis Transmembrane Conductance Regulator
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.