Article
Primary Ciliary Dyskinesia Patient-Specific hiPSC-Derived Airway Epithelium in Air-Liquid Interface Culture Recapitulates Disease Specific Phenotypes In Vitro.
Cells - 24 May 2023
von Schledorn Laura, Puertollano Martín David, Cleve Nicole, Zöllner Janina, Roth Doris, Staar Ben Ole, Hegermann Jan, Ringshausen Felix C, Nawroth Janna, Martin Ulrich, Olmer Ruth
Abstract excerpt
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...
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