Mapping SP-C co-chaperone binding sites reveals molecular consequences of disease-causing mutations on protein maturation

Abstract

Interstitial Lung Disease (ILD)-associated mutations in surfactant protein C (SP-C) render the protein prone to aggregation. Here, the authors reveal their impact on protein maturation, provide insights into recognition of aggregation prone regions by chaperones, and address the autosomal dominant nature of ILD mutants.