Prion Biology
Abstract: My research focuses on the [URE3] prion in yeast. Prions are the infectious agents that cause the mammalian transmissible spongiform encephalopathies, the most notorious of which is Mad Cow Disease. Prions are infectious proteins that induce the formation of amyloid fibers. Amyloid fibers are also implicated in the progression of diseases such as Alzheimer’s, Parkinson’s, Huntington’s, type II diabetes, and cystic fibrosis, although none of these diseases are caused by prions. Yeast do not get any of the diseases associated with prions and amyloids – they are altered in other ways -- and yeast prions cannot infect humans, making them perfectly safe to work with. Thus yeast prions provide a safe and powerful means of investigating the molecular mechanisms of prion infectivity and amyloid formation. In my lab at MUW we are investigating how other gene products, such as those of the so-called heat shock genes which encode chaperone proteins, interact with the [URE3] prion to either facilitate or inhibit amyloid formation. Our goal is to do a systematic search of the yeast genome to identify gene products that might be targeted in treatments designed to slow or prevent the progression of the human amyloid-associated diseases.
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