Recent studies have shown that autophagy mitigates the pathological effects of
proteinopathies in the liver, heart, and skeletal muscle but this has not been investigated for
proteinopathies that affect the lung. This may be due at least in part to the lack of an animal
model robust enough for spontaneous pathological effects from proteinopathies even
though several rare proteinopathies, surfactant protein A and C deficiencies, cause severe
pulmonary fibrosis. In this report we show that the PiZ mouse, transgenic for the common …