Prions and prion diseases: a review.

Abstract

The name “príon” is given to an altered form of a protein present at the surface of neurons. Prions are remarkable for been able to induct their normal counterpart to assume the pathological configuration on a kind of chain reaction, with the possibility of trasmission between subjects and even through different species. Prions can cause a series of neurodegenerative disorders affecting humans (Creutzfeldt-Jakob disease, Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia, kuru), and animals such cattle (bovine spongiform encephalopathy or “mad cow disease”), deer (chronic wasting disease) and sheep (scrapie). On this article, we give a general view about the nature of prions, followed by a brief discussion of the human diseases caused by prions.

Keywords: prion, Creutzfeld-Jakob disease, transmissible spongiform encephalopathy, Gerstmann-Straussler-Scheiker syndrome, bovine spongiform encephalopathy.