Browsing by Subject ABCA3 proteins
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Surfactants produced by type II alveolar epithelial cells (AT2 cells) are usually present in inclusion organelles called lamellar bodies (LBs). The ATP-binding cassette subfamily A member 3 (ABCA3) transporter primarily exists in AT2 cells and is generally considered to be one of the critical regulators of biogenesis of LBs and surfactant metabolism in the lungs [1,2,3,4]. ABCA3 mutations are the most common cause of congenital surfactant dysfunction disorders (CSDDs), resulting in fatal neonatal respiratory distress and pediatric or adult interstitial lung disease [3, 5,6,7]. |
