Prion diseases are deadly neurodegenerative disorders in humans and animals that are characterized by misfolded forms of prion protein (PrP). Development of effective treatments has been hampered by the lack of good experimental models. In a new study published in The American Journal of Pathology, researchers describe the distinct stages of prion disease in the mouse retina and define an experimental model in which to specifically test therapeutic approaches. The article is tilted “Temporal Resolution of Misfolded Prion Protein Transport, Accumulation, Glial Activation, and Neuronal Death in the Retinas of Mice Inoculated with Scrapie.” "This work was done in collaboration with the USDA Agricultural Research Service and is an excellent example of how animal disease research can be leveraged to benefit human health," commented Heather West Greenlee, Ph.D., an Associate Professor in the Department of Biomedical Sciences at the Iowa State University College of Veterinary Medicine (Ames IA). "It provides important insights into the timeline for key pathologic milestones of prion disease in the retina and a model to study mechanisms of disease progression and evaluate therapeutic interventions." The study used an experimental mouse model of the prion disease scrapie to determine the temporal relationship between the transport of misfolded prion protein (PrPSc) from the brain to the retina, the accumulation of PrPSc in the retina, the inflammatory response of the surrounding retinal tissue, and the loss of neurons. It is believed that transmissible spongiform encephalopathy (TSE) progression depends on the spread of misfolded protein from one central nervous system structure to another.
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