Article
High-Content Screening Identifies Cyclosporin A as a Novel ABCA3-Specific Molecular Corrector.
American journal of respiratory cell and molecular biology - 1 Apr 2022
Forstner Maria, Lin Sean, Yang Xiaohua, Kinting Susanna, Rothenaigner Ina, Schorpp Kenji, Li Yang, Hadian Kamyar, Griese Matthias
Abstract excerpt
ABCA3 (ATP-binding cassette subfamily A member 3) is a lipid transporter expressed in alveolar type II cells and localized in the limiting membrane of lamellar bodies. It is crucial for pulmonary surfactant storage and homeostasis. Mutations in the ABCA3 gene are the most common genetic cause of respiratory distress syndrome in mature newborns and of interstitial lung disease in children. Apart from lung...
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