The leading cause of death in infants born prematurely is respiratory distress syndrome. It is caused by deficiency in a fat-protein complex known as lung surfactant, which is critical for optimal gas exchange in the lung. LPCAT1 is a recently identified mouse lung protein predicted, based on in vitro assays, to be involved in the generation of surfactant. Now, John Shannon and colleagues, at Cincinnati Children's Hospital Medical Center, have demonstrated that LPCAT1 has a crucial role in the generation of surfactant in vivo in mice and that the activity of this protein must be maximal for the transition from the womb to air breathing. They therefore speculate that decreased LPCAT1 expression, as a result of mutations in the gene responsible for making this protein, might underlie the fatal respiratory distress syndrome observed in a subset of newborn infants.
TITLE: LPCAT1 regulates surfactant phospholipid synthesis and is required for transitioning to air breathing in mice
AUTHOR CONTACT:
John M. Shannon
Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
Phone: 513.636.2938; Fax: 513.636.7868; E-mail: John.Shannon@cchmc.org .
View this article at: http://www.jci.org/articles/view/38061?key=6046593af9d76bdabca5
Journal of Clinical Investigation