ABSTRACT
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are fatal neurological disorders that include Creutzfeldt–Jakob disease (CJD) in humans, scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle, chronic wasting disease (CWD) in cervids, transmissible mink encephalopathy (TME) in mink, feline spongiform encephalopathy (FSE) in cats, and exotic ungulate encephalopathy (EUE) in zoo animals such as kudu, nyala, gemsbok, eland, and oryx. This chapter describes laboratory models used for analyzing prion infections. The laboratory models for prion infections are divided into in vitro models, cellular models, and animal models. In all three cases, prion infection is diagnosed by detection of PrPSc. The representative in vitro model for prion infection is protein misfolding cyclic amplification (PMCA). Several prion-susceptible cell lines have been exploited to generate cell-culture models for prion infection. Studies using cell lines suggest that PrPC is not the sole determinant for prion susceptibility.
