부산대학교 도서관

Prions are infectious agents that cause a devastating neurodegenerative disease in both humans and animals. Unlike other rodents, bank vole (Myodes glareolus, BV) is susceptible to prions from a diverse range of species, including humans. There are two lines of BVs, one homozygous for methionine (BV109M) and the other for isoleucine at codon 109 (BV109I) of prion protein (PrP). Here, we established neuronal cell lines from embryonic brain of BV109I mouse that was immortalized by an introduction of plasmid DNA encoding for SV40-T antigen. Bv109I mice develop age-dependent signs of spontaneous neurologic illness. The established BV cell lines infected with 22L scrapie strain (BV-22L) showed a replication and an accumulation of disease-associated forms of the PrP. BV-22L infected neuronal cells were then intracerebrally inoculated into ICR mice showing clinical symptoms of disease after ~147 days post-inoculation. We also confirmed that various prion strains, including ME7, 263K, CWD and 22L, were successfully infected into BV neuronal cells. The newly established BV cell models may enhance our understanding on the cellular mechanisms of prion replication and can be a useful tool for the study of the pathogenic mechanisms of prion diseases.