Article
Divergent pathways of surfactant protein C maturation for disease-associated isoforms.
The Journal of biological chemistry - 1 Mar 2026
Bui Sarah, Reineberg Anamarie, Jones Dakota, Na Cheng-Lun, Kitzmiller Joseph, Rodriguez Luis R, Murthy Aditi, Iyer Swati, Cooper Charlotte, Chroneos Rea, Tomer Yaniv, Mulugeta Surafel, Weaver Timothy E, Kotton Darrell N, Alysandratos Konstantinos-Dionysios, Whitsett Jeffrey A, Beers Michael F
Abstract excerpt
Surfactant protein C (SP-C), a hydrophobic protein exclusively synthesized and secreted by alveolar type II (AT2) cells, is important for reducing alveolar surface tension in the distal lung. Chronic interstitial pulmonary diseases have been associated with SFTPC mutations. However, a detailed understanding of SP-C maturation in the secretory pathway and disruptions caused by mutations has remained incomplete....
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