CareLit Fachartikel
Cellular mechanisms of interstitial lung disease in children caused by mutations of the ABCA3-transporter
N Weichert, E Kaltenborn, A Hector, M Woischnik, S Moslavac, M Griese · Pneumologie · 2010 · Heft 01
Dokument
1168078
CareLit-ID
Jahr
2010
Publikation
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Zusammenfassung
Introduction: ABCA3 protein is an ABC lipid transporter found in alveolar type-II cells (ATII), where it localizes to the outer membrane of lamellar bodies (LB) which store surfactant. Mutations in the ABCA3 gene are the most common known genetic cause of respiratory distress syndrome in newborns and of late onset interstitial lung disease (ILD) in children. Even though clinical experience suggests a link between the late onset of genetic ILD and exposure to outside stress factors, such as respiratory infections and cigarette smoke, contributing mechanisms remain unknown. We have studied how the clinically relev…
Schlagworte
A549 Cells
Annexin V
Caspase 3
Apoptosis
Axis
Pneumologie
