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von Schledorn, L.* ; Puertollano Martín, D.* ; Cleve, N.* ; Zöllner, J.* ; Roth, D. ; Staar, B.O.* ; Hegermann, J.* ; Ringshausen, F.C.* ; Nawroth, J. ; Martin, U.* ; Olmer, R.*

Primary ciliary dyskinesia patient-specific hiPSC-derived airway epithelium in air-liquid interface culture recapitulates disease specific phenotypes in vitro.

Cells 12:19 (2023)

DOI

PMC

Open Access Gold as soon as Publ. Version/Full Text is submitted to ZB.

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Primary ciliary dyskinesia (PCD) is a rare heterogenic genetic disorder associated with perturbed biogenesis or function of motile cilia. Motile cilia dysfunction results in diminished mucociliary clearance (MCC) of pathogens in the respiratory tract and chronic airway inflammation and infections successively causing progressive lung damage. Current approaches to treat PCD are symptomatic, only, indicating an urgent need for curative therapeutic options. Here, we developed an in vitro model for PCD based on human induced pluripotent stem cell (hiPSC)-derived airway epithelium in Air-Liquid-Interface cultures. Applying transmission electron microscopy, immunofluorescence staining, ciliary beat frequency, and mucociliary transport measurements, we could demonstrate that ciliated respiratory epithelia cells derived from two PCD patient-specific hiPSC lines carrying mutations in DNAH5 and NME5, respectively, recapitulate the respective diseased phenotype on a molecular, structural and functional level.

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Publication type Article: Journal article

Document type Scientific Article

Corresponding Author

Keywords Pcd ; Air-liquid Interface Culture ; Human Induced Pluripotent Stem Cells; Pluripotent Stem-cells; Scalable Expansion; Beat Pattern; Mutations; Generation; Defects; Model

ISSN (print) / ISBN 2073-4409

e-ISSN 2073-4409

Journal Cells

Quellenangaben Volume: 12, Issue: 11, Article Number: 19

Publisher MDPI

Publishing Place Basel

Non-patent literature Publications

Institute(s) Helmholtz Pioneer Campus (HPC)
Institute of Biological and Medical Imaging (IBMI)

Grants German Center for Lung Research (DZL)