Article
ABCA3 is critical for lamellar body biogenesis in vivo.
The Journal of biological chemistry - 17 Aug 2007
Cheong Naeun, Zhang Huayan, Madesh Muniswamy, Zhao Ming, Yu Kevin, Dodia Chandra, Fisher Aron B, Savani Rashmin C, Shuman Henry
Abstract excerpt
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...
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