Evidence map›Paper›PMID 41064994›Full record

ArticleJournal of cell science2025

Characterisation of a primary ciliary dyskinesia model generated from BMI1-transduced basal epithelial cells.

Melis T Dalbay, Eriomina Shahaj, Ileana Guerrini, Dani Do Hyang Lee, Anna Straatman-Iwanowska, Hannah M Mitchison, Deborah L Baines, Robert A Hirst, Claire Hogg, Christopher O'Callaghan and 1 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Journal of cell science, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. DNAAF19-RUVBL1/2 complex recruits multiple adaptors to promote dynein arm assembly.Proceedings of the National Academy of Sciences of the United States of America · 2026
    Article
  2. Preclinical human models of primary ciliary dyskinesia.European respiratory review : an official journal of the European Respiratory Society · 2026
    Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

11 authors.

Melis T DalbayDepartment of Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.ORCID 0000-0002-2691-5941
Eriomina ShahajDepartment of Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.ORCID 0000-0001-5428-0405
Ileana GuerriniDepartment of Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Dani Do Hyang LeeDepartment of Infection, Immunity & Inflammation, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Anna Straatman-IwanowskaCentre for Primary Ciliary Dyskinesia Diagnosis and Research, Department of Respiratory Sciences, University of Leicester, Leicester LE1 7HB, UK.
Hannah M MitchisonDepartment of Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Deborah L BainesInstitute for Infection and Immunity , St George's University, University of London, London EC1V 0HB, UK.
Robert A HirstCentre for Primary Ciliary Dyskinesia Diagnosis and Research, Department of Respiratory Sciences, University of Leicester, Leicester LE1 7HB, UK.
Claire HoggPCD Diagnostic Centre , Royal Brompton and Harefield NHS Trust, London SW3 6NP, UK.
Christopher O'CallaghanDepartment of Infection, Immunity & Inflammation, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.
Stephen L HartDepartment of Genetics and Genomic Medicine, UCL Great Ormond Street Institute of Child Health, University College London, London WC1N 1EH, UK.ORCID 0000-0001-8254-376X

Funding

National Institute for Health Research Great Ormond Street Hospital Biomedical Research CentreNewlife - The Charity for Disabled ChildrenNIHR Great Ormond Street Hospital Biomedical Research CentreUniversity College London
6 · The paper itself

Abstract

Primary ciliary dyskinesia (PCD) is a rare genetic respiratory disorder caused by a reduction in cilia number or cilia dysmotility. Cilia dysmotility leads to breathing difficulties, concurrent infections and severe lung damage if not treated, with no therapies currently available. Improved airway epithelial cell models that mimic the disease phenotype are required for development of new therapeutics, as current models have limited potential of self-renewal in vitro. Here, we describe a human PCD cell model created by lentiviral transduction of airway basal epithelial cells with the BMI1 gene, a regulator of senescence. We report that the cells retain their proliferation and differentiation capacity for at least 19 passages and recapitulate the disease phenotype with immotile cilia lacking DNAH5 and other outer dynein arm proteins. Characterisation of the ion transport properties of these PCD cells grown at an air-liquid interface showed lower activity of the Na+ channel ENaC and enhanced CFTR activity compared to non-PCD cells, which might be linked to ciliary immotility. Our study provides a robust PCD model for therapeutic studies, opening new avenues to investigate the molecular mechanisms of this disease.

Indexed as

Ciliary Motility DisordersEpithelial CellsModels, BiologicalPolycomb Repressive Complex 1Cell DifferentiationCell ProliferationCiliaCystic Fibrosis Transmembrane Conductance RegulatorHumansTransduction, GeneticBMI1 protein, humanCystic Fibrosis Transmembrane Conductance RegulatorPolycomb Repressive Complex 1Airway cell culture modelsENaCPCD

Identifiers

PMID41064994
PMCPMC12633737

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Registered trials

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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 OpenQuestion graph.