Abstract

Prion diseases encompass a family of fatal neurodegenerative conditions that have gained notoriety due to their transmissible nature. Public awareness of these diseases is often limited to the variant of Creutzfeldt Jakob Disease that emerged following bovine spongiform encephalopathy (mad cow disease) contaminated meat entering the food supply. However, these diseases more commonly arise sporadically or are hereditary. Despite decades of research there are currently no effective treatments and few in development. Likewise, an understanding of the pathogenesis within the human brain still eludes us and translating the findings from animal models into knowledge of human disease has been hindered by a lack of human model systems. The ability to investigate prion diseases in living human brain tissue presented itself following the development of human cerebral organoids. Cerebral organoids are spheres of brain tissue that can be grown on demand in the laboratory from stem cells. This presentation will outline how the use of human cerebral organoids has advanced our examination of neurodegeneration during prion diseases and what they might add to the search for a therapy.