{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,15]],"date-time":"2026-10-15T19:21:55Z","timestamp":1792092115858,"version":"4.4.0"},"reference-count":54,"publisher":"The Company of Biologists","issue":"20","license":[{"start":{"date-parts":[[2025,10,31]],"date-time":"2025-10-31T00:00:00Z","timestamp":1761868800000},"content-version":"vor","delay-in-days":16,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100000871","name":"Newlife \u2013 The Charity for Disabled Children","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100000871","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100019256","name":"National Institute for Health Research Great Ormond Street Hospital Biomedical Research Centre","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100019256","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100000765","name":"University College London","doi-asserted-by":"crossref","id":[{"id":"10.13039\/501100000765","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,10,15]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>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\u2013liquid 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.<\/jats:p>","DOI":"10.1242\/jcs.263886","type":"journal-article","created":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T10:02:29Z","timestamp":1760004149000},"source":"Crossref","is-referenced-by-count":4,"title":["Characterisation of a primary ciliary dyskinesia model generated from\n                    <i>BMI1<\/i>\n                    -transduced basal epithelial cells"],"prefix":"10.1242","volume":"138","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2691-5941","authenticated-orcid":false,"given":"Melis T.","family":"Dalbay","sequence":"first","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health, University College London 1 Department of Genetics and Genomic Medicine , , London WC1N 1EH ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5428-0405","authenticated-orcid":false,"given":"Eriomina","family":"Shahaj","sequence":"additional","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health, University College London 1 Department of Genetics and Genomic Medicine , , London WC1N 1EH ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ileana","family":"Guerrini","sequence":"additional","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health, University College London 1 Department of Genetics and Genomic Medicine , , London WC1N 1EH ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dani Do Hyang","family":"Lee","sequence":"additional","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health, University College London 2 Department of Infection, Immunity & Inflammation , , London WC1N 1EH ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Straatman-Iwanowska","sequence":"additional","affiliation":[{"name":"Centre for Primary Ciliary Dyskinesia Diagnosis and Research 3 , Department of Respiratory Sciences , , Leicester LE1 7HB ,","place":["UK"]},{"name":"University of Leicester 3 , Department of Respiratory Sciences , , Leicester LE1 7HB ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hannah M.","family":"Mitchison","sequence":"additional","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health, University College London 1 Department of Genetics and Genomic Medicine , , London WC1N 1EH ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah L.","family":"Baines","sequence":"additional","affiliation":[{"name":", St George's University, University of London 4 Institute for Infection and Immunity , London EC1V 0HB ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert A.","family":"Hirst","sequence":"additional","affiliation":[{"name":"Centre for Primary Ciliary Dyskinesia Diagnosis and Research 3 , Department of Respiratory Sciences , , Leicester LE1 7HB ,","place":["UK"]},{"name":"University of Leicester 3 , Department of Respiratory Sciences , , Leicester LE1 7HB ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"Hogg","sequence":"additional","affiliation":[{"name":", Royal Brompton and Harefield NHS Trust 5 PCD Diagnostic Centre , London SW3 6NP ,","place":["UK"]},{"name":"Imperial College London 6 Paediatric Respiratory Medicine , , London SW3 6NP ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher","family":"O'Callaghan","sequence":"additional","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health, University College London 2 Department of Infection, Immunity & Inflammation , , London WC1N 1EH ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8254-376X","authenticated-orcid":false,"given":"Stephen L.","family":"Hart","sequence":"additional","affiliation":[{"name":"UCL Great Ormond Street Institute of Child Health, University College London 1 Department of Genetics and Genomic Medicine , , London WC1N 1EH ,","place":["UK"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2025,10,31]]},"reference":[{"key":"2025103107251005400_JCS263886C1","doi-asserted-by":"publisher","first-page":"2788","DOI":"10.1091\/mbc.E15-01-0018","article-title":"DRC3 connects the N-DRC to dynein g to regulate flagellar waveform","volume":"26","author":"Awata","year":"2015","journal-title":"Mol. 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