Reyes-Pablo A, Mena LR, Luna-Muñoz J, García SF

Language: Spanish
References: 55
Page: 597-602
PDF size: 99.74 Kb.

ABSTRACT

Introduction: At present time, it is known that prion diseases or transmissible spongiform encephalopaties (TSE), represent a group of neurodegenerative pathologies of the central nervous system, that affect humans as well as animals. The least rare of these is Creutzfeldt-Jakob disease (CJD). In 1998 the first case of Gerstman-Sträussler-Scheinker (GSS) of familiar type was reported (a case by million families). The bovine spongiform encephalopaty (BSE) - better known as “crazy cows disease” - is attributed to the consumption of contaminated products with affected cows meat. The prion disease can appear like a genetic disorder, infectious or sporadic, which involve modifications of the prion protein (PrP), a normal component of the mammal cells. Recently it has been suggested that cellular prion protein (PrPc) acts like a metaloprotein, and has an intrinsic tendency to adopt some structural characteristics of the Scrapie prion protein (PrPsc).

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