ABCA3 inactivation in mice causes respiratory failure, loss of pulmonary surfactant, and depletion of lung phosphatidylglycerols⃞
ML Fitzgerald, R Xavier, KJ Haley, R Welti… - Journal of lipid …, 2007 - ASBMB
The highly branched mammalian lung relies on surfactant, a mixture of phospholipids,
cholesterol, and hydrophobic proteins, to reduce intraalveolar surface tension and prevent
lung collapse. Human mutations in the ABCA3 transporter have been associated with
childhood respiratory disease of variable severity and onset. Here, we report the generation
of Abca3 null mice, which became lethargic and cyanotic and died within 1 h of birth. Tissue
blots found ABCA3 expression was highest in lung but was also detectable in other tissues …
cholesterol, and hydrophobic proteins, to reduce intraalveolar surface tension and prevent
lung collapse. Human mutations in the ABCA3 transporter have been associated with
childhood respiratory disease of variable severity and onset. Here, we report the generation
of Abca3 null mice, which became lethargic and cyanotic and died within 1 h of birth. Tissue
blots found ABCA3 expression was highest in lung but was also detectable in other tissues …
