Prion protein: Difference between revisions

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==Prion diseases==
==Prion diseases==
The naturally ocuring prion diseases include Creutzfeldt Jakob disease (CJD) in people, bovine spongiform encephalopathy (BSE) commonly known as "mad cow" disease, scrapie in sheep and goats, and chronic wasting disease in [http://www.wikipedia.org/wiki/Cervidae cervids]. In all cases ''post mortem'' analysis of brain tissue is characterized by aggregates of PrP<sup>Sc</sup>.
The naturally ocuring prion diseases include [http://en.wikipedia.org/wiki/Creutzfeldt-Jakob_disease Creutzfeldt-Jakob disease] (CJD) in people, [http://en.wikipedia.org/wiki/Bovine_spongiform_encephalopathy bovine spongiform encephalopathy] (BSE) commonly known as "mad cow" disease, [http://en.wikipedia.org/wiki/Scrapie scrapie] in sheep and goats, and [http://en.wikipedia.org/wiki/Chronic_wasting_disease chronic wasting disease] in deer. In all cases ''post mortem'' analysis of brain tissue is characterized by aggregates of PrP<sup>Sc</sup>.
The sporadic, genetic and infectious etiologies of prion diseases can be explained by a simple protein-based model in which PrP<sup>C</sup> is converted into PrP<sup>Sc</sup> that in turn initiates an autocatalytic refolding cascade of PrP<sup>C</sup> in a template-dependent manner.
The sporadic, genetic and infectious etiologies of prion diseases can be explained by a simple protein-based model in which PrP<sup>C</sup> is converted into PrP<sup>Sc</sup> that in turn initiates an autocatalytic refolding cascade of PrP<sup>C</sup> in a template-dependent manner.


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Kurt Giles, Jaime Prilusky, Eran Hodis, Claudio Garutti, Michal Harel, Joel L. Sussman