Abnormal Expression of Proteolytic Stress-Related Proteins and Protective Effect of Fibrinolytic Enzymes in Prion Diseases.

ABSTRACT: Prion diseases are fatal, irreversible, and infectious neurodegenerative diseases caused by proteinase K-resistant prion protein (PrP^Sc). Against PrP^Sc, several endogenous proteases involved in cellular degradation mechanisms can be activated to remove PrP^Sc. However, since PrP^Sc shows proteinase K resistance, we presumed that undegradable PrP^Sc induces positive feedback on the overactivation of the cellular degradation mechanisms and is correlated with proteolytic stress and exacerbation of the progression of prion diseases. We investigated the expression pattern of proteolytic stress-related proteins in the brains of ME7 scrapie-infected mice at 7 months postinfection and sporadic Creutzfeldt-Jakob disease (CJD) patients using wester

SUBMITTER: Kim YC 

PROVIDER: S-EPMC12017092 | biostudies-literature | 2025

REPOSITORIES: biostudies-literature