Transmissible Spongiform Encephalopathies

Transmissible spongiform encephalopathies (TSEs) are a group of rare, fatal, neurodegenerative diseases caused by misfolded prion proteins. They include Creutzfeldt-Jakob Disease (CJD), Kuru, Gerstmann-Sträussler-Scheinker Syndrome (GSS), and Fatal Familial Insomnia (FFI). TSEs are notorious for their long incubation periods, often ranging from months to decades, and present as slowly progressive dementia, seizure, and ataxia. While there is no known cure or preventive measure for TSEs, early diagnosis may help manage symptoms and slow disease progression. There is currently an immense interest in the development of new and improved animal model systems to further our understanding of TSEs and to assist in the development of new treatments.