“…ABCA3 is expressed predominantly at the limiting membrane of the lamellar bodies in lung alveolar type II cells and is proposed to be a surfactant lipid transporter (20,36). Exogenous expression of ABCA3 in cultured cells promotes lipid uptake into intracellular vesicles that generate lamellar body-like vesicles (7,18,21). ABCA3 deficiency in human and mice leads to decreased phosphatidylcholine and phosphatidylglycerol in surfactant, dysgenesis of lamellar bodies, and respiratory distress (1,3,8,11,12,27).…”