ABCA3 mutations influence the stress level and immunological function of lung epithelial A549 cells

Einleitung: ABCA3 is an ABC-transporter in the outer membrane of lamellar bodies (LBs) in alveolar epithelial type II cells (ATII) involved in the surfactant lipid transport. Mutations in the ABCA3 gene have been associated with genetic interstitial lung disease (ILD). Next to being essential for surfactant secretion, ATII are an active part of the lung immunological system and involved in lung repair. We investigate how ABCA3 mutations influence ATII cell homeostasis, primary the stress level and immunological function, and potential involvement of ATII cells in epithelial-mesenchymal transition (EMT). Methoden…