[HTML][HTML] ABCA3 as a lipid transporter in pulmonary surfactant biogenesis
N Ban, Y Matsumura, H Sakai, Y Takanezawa… - Journal of Biological …, 2007 - ASBMB
ABCA3 protein is expressed predominantly at the limiting membrane of the lamellar bodies
in alveolar type II cells, and mutations in the ABCA3 gene cause lethal respiratory distress in
newborn infants. To investigate the function of ABCA3 protein, we generated Abca3-
deficient mice by targeting Abca3. Full-term Abca3–/–newborn pups died within an hour
after birth because of acute respiratory failure. Ultrastructural analysis revealed abnormally
dense lamellar body-like organelles and no normal lamellar bodies in Abca3–/–alveolar …
in alveolar type II cells, and mutations in the ABCA3 gene cause lethal respiratory distress in
newborn infants. To investigate the function of ABCA3 protein, we generated Abca3-
deficient mice by targeting Abca3. Full-term Abca3–/–newborn pups died within an hour
after birth because of acute respiratory failure. Ultrastructural analysis revealed abnormally
dense lamellar body-like organelles and no normal lamellar bodies in Abca3–/–alveolar …