Article
Expression of a human surfactant protein C mutation associated with interstitial lung disease disrupts lung development in transgenic mice.
The Journal of biological chemistry - 26 Dec 2003
Bridges James P, Wert Susan E, Nogee Lawrence M, Weaver Timothy E
Abstract excerpt
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...
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