[HTML][HTML] ABCA3 is critical for lamellar body biogenesis in vivo
N Cheong, H Zhang, M Madesh, M Zhao, K Yu… - Journal of Biological …, 2007 - ASBMB
Mutations in ATP-binding cassette transporter A3 (human ABCA3) protein are associated
with fatal respiratory distress syndrome in newborns. We therefore characterized mice with
targeted disruption of the ABCA3 gene. Homozygous Abca3–/–knock-out mice died soon
after birth, whereas most of the wild type, Abca3+/+, and heterozygous, Abca3+/–, neonates
survived. The lungs from E18. 5 and E19. 5 Abca3–/–mice were less mature than wild type.
Alveolar type 2 cells from Abca3–/–embryos contained no lamellar bodies, and expression …
with fatal respiratory distress syndrome in newborns. We therefore characterized mice with
targeted disruption of the ABCA3 gene. Homozygous Abca3–/–knock-out mice died soon
after birth, whereas most of the wild type, Abca3+/+, and heterozygous, Abca3+/–, neonates
survived. The lungs from E18. 5 and E19. 5 Abca3–/–mice were less mature than wild type.
Alveolar type 2 cells from Abca3–/–embryos contained no lamellar bodies, and expression …