The Eyes Have it: A Functional Role for Prion Protein

https://www.niaid.nih.gov/news-events/prion-role-light?utm_campaign=+62411034&utm_content=&utm_medium=email&utm_source=govdelivery&utm_term= In the early 1980s, scientists identified clumps of abnormal, misfolded prion protein in mammals as the cause of brain-wasting diseases, now called prion diseases. Human prion diseases include Creutzfeldt-Jakob disease, fatal familial insomnia and kuru; animal prion diseases include scrapie in sheep and chronic wasting disease in cervids. Since identifying prion protein, researchers have struggled to answer: What do they do? A new study published in iScience from NIAID scientists at Rocky Mountain Laboratories in Hamilton, Montana, and colleagues provides details of how prion protein functions in the retina of mouse eyes, helping them respond to light. The researchers believe the prion protein must be present for rods and cones to function normally. The team hopes these findings help colleagues who study prion diseases better understand what might occur in humans when natural forms of prion protein are therapeutically removed. New treatment strategies for prion diseases focus on using drugs that remove natural prion protein to eliminate the potential for misfolding and clumping. The findings also could extend to other protein-related neurodegenerative diseases, such as Alzheimer’s (amyloid beta protein) and Parkinson’s diseases (alpha synuclein protein).