Using our mini-brains: cerebral organoids as an improved cellular model for human prion disease

The most common form of human prion disease is sporadic Creutzfeldt-Jakob disease (CJD), occurring in 1–2 people per million per year. Progression of prion diseases results in protease resistant prion protein (PrP) deposition, astrogliosis, and neuronal loss with spongiosis. Animal models for human prion disease have been instrumental to our understanding of the disease; they demonstrate many of the pathological features found in human disease including PrP deposition, protease resistant PrP, spongiform change, astrogliosis and death. [...]one general difficulty with genetic neurodegenerative diseases, such as the above-mentioned study, is that following differentiation, disease progression may take a longer time to manifest than the culture system will allow.