Mutations in the SFTPC gene lead to misfolding of the encoded protein, surfactant protein C (SP-C). Molecular chaperones play a key role in detecting misfolded SP-C and directing the protein to the proteasome via the endoplasmic reticulum-associated degradation (ERAD) pathway. Under certain conditions (e.g. viral infection) misfolded SP-C may accumulate in the cytosol where it enters the autophagy pathway resulting in degradation in the lysosome. Failure at any point in these two quality-control pathways (ERAD and autophagy) can lead to accumulation of cytotoxic protein aggregates, type II cell injury and, ultimately, lung disease. The goal of this project is to identify the molecular components of these cytoprotective pathway(s) and design reagents that enhance rapid elimination of mutant SP-C in type II epithelial cells.