Priones y enfermedades espongiformes transmisibles

Abstract

Transmissible spongiform encephalopathies (TSE) are a group of lethal neurodegenerative diseases in humans and animals, related to a new infectious agent: the prión. These diseases are the result of a conformational change in the PrP protein into a proteases-resistant protein (PrPSc). In humans, these diseases can be divided into three groups: infectious TSE, sporadic TSE and familial or genetic TSE. In 1996, authorities in the United Kingdom discovered the variant of Creutzfeldt-Jakob disease, the human form of the bovine spongiform encephalopathy which can be acquired through ingestion of contaminated meat. Nowadays, there is not an effective drug to stop or delay the damages produced by prións, and this represents a big challenge for the scientific community.