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[PubMed 9921266] 2287

Accordingly, several cellular hallmarks of proteotoxic stress have been identified, including dysregulated mTOR signaling and autophagy, compartment-specific unfolded protein response in mitochondria (UPR mt ), and in the endoplasmic reticulum (UPR ER )

This alteration is characterized by the presence of necrotic hepatocytes, hepatocytes exhibiting poor staining, and ruptures in the walls of hepatic vessels

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CPFE can be particularly challenging to diagnose because the two conditions partially mask each other's effects on pulmonary function tests, making results appear more normal than the actual level of damage warrants