Article
ABCA3 gene mutations in newborns with fatal surfactant deficiency.
The New England journal of medicine - 25 Mar 2004
Shulenin Sergey, Nogee Lawrence M, Annilo Tarmo, Wert Susan E, Whitsett Jeffrey A, Dean Michael
Abstract excerpt
BACKGROUND: Pulmonary surfactant forms a lipid-rich monolayer that coats the airways of the lung and is essential for proper inflation and function of the lung. Surfactant is produced by alveolar type II cells, stored intracellularly in organelles known as lamellar bodies, and secreted by exocytosis. The gene for ATP-binding cassette transporter A3 (ABCA3) is expressed in alveolar type II cells, and the protein...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.