Pathogenic mechanisms in prion disease

Published on August 5, 2014   32 min

Other Talks in the Series: The Genetic Basis of Neurological Disorders

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So I'm Giovanna Mallucci. I'm program leader the MRC Toxicology Unit and professor of neuroscience. And I'm also a consultant neurologist at the specialist interested dimensions and Neurodegeneration. And I'm going to talk to you about pathogenic mechanisms in prion disease and also their relevance for other neurodegenerative diseases, particularly dementia.
So in this talk, I'm going to tell you about the work we've done which led to the discovery that early prion neurodegeneration can be reversed. I'm going to tell you about the discovery of the molecules and pathways that underlie this process, about pharmacological treatment that cures prion disease, and relevance to other neurodegenerative disorders.
So just to introduce the neurodegeneration diseases as a group, these include Alzheimer's, Parkinson's, Huntington's, ALS, and the rarest of all are the prion diseases. The top group-- Alzheimer's, Parkinson's, and vascular dementia and other forms of dementia-- threaten to be the second commonest cause of death in developed world by 2040. And this is a massive global crisis at the moment that led to the G8 summit on dementia, calling for a cure for these disorders by 2025. At the moment, they cost 1% of the world's GDP. So there's a huge economic, human, and medical burden on societies of these diseases. And although prion are the rarest of these disorders, they're very important mechanistically as they give us insight into mechanisms. And the alternate name for all of those diseases is the protein misfolding disorders.