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

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Neurodegenerative diseases are an ever-increasing burden in an aging society. Currently no cure is available for any of these diseases and treatment is based on managing symptoms. Despite many candidate therapeutics demonstrating promise in animal models, none has yet shown effcacy in human trials. It is self-evident that humans are differ-ent from the animals used to model our diseases, especially models that have been highly manipulated to generate a disease in an animal that does not naturally have such a disease. These differences are likely the reason for the failures of drug candidates in human trials but, until re-cently, human models of neurodegenerative diseases were lacking. The development of the human cerebral organoid model, by differentiating three-dimensional human neuronal tissue from pluripotent stem cells, represents a significant advance in studying human brain diseases. Ce-rebral organoids have been used to model Alzheimer’s disease, Parkin-son’s disease, Down’s syndrome dementia and we have now shown they can be infected with human prions creating a new model of human prion diseases.
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