[HTML][HTML] Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice

JP Bridges, SE Wert, LM Nogee, TE Weaver - Journal of Biological …, 2003 - ASBMB
Surfactant Protein C (SP-C) is a secreted transmembrane protein that is exclusively
expressed by alveolar type II epithelial cells of the lung. SP-C associates with surfactant
lipids to reduce surface tension within the alveolus, maintaining lung volume at end
expiration. Mutations in the gene encoding SP-C (SFTPC) have recently been linked to
chronic lung disease in children and adults. The goal of this study was to determine whether
a disease-linked mutation in SFTPC causes lung disease in transgenic mice. The SFTPC …