Enfermedades priónicas: historia, diversidad e importancia socioeconómica como paradigma de las Enfermedades Raras

Jorge M. Charco; Tomás Barrio; Hasier Eraña

doi:10.12795/araucaria.2021.i46.21

Authors

Jorge M. Charco Centro de investigación cooperativa en biociencias (CIC bioGUNE). ATLAS Molecular Pharma S
Tomás Barrio Centro de investigación cooperativa en biociencias (CIC bioGUNE). ATLAS Molecular Pharma S.L.
Hasier Eraña Centro de investigación cooperativa en biociencias (CIC bioGUNE). ATLAS Molecular Pharma S.L.

DOI:

https://doi.org/10.12795/araucaria.2021.i46.21

Keywords:

Prions, Rare diseases, prions diseases

Abstract

Rare disease are those pathologies that affect a reduced proportion of the population (less than 50 cases per 100 000 people). For this reason, the research on their causes and mechanisms, which is essential to find a way to treat or prevent them, is insufficient. This causes that the patients report a lack of coverage by the health system and the social discrimination that suffering one of these pathologies entails. Among rare diseases, we find the so-called prion diseases or transmissible spongiform encephalopathies. Although they are relatively well-known due to the health crisis provoked by the “mad cow disease” at the end of the last century, there are still many uncertainties about these disorders that affect both animals and humans. This monograph aims at bringing to light the fascinating history and the diversity of prion diseases, which shook the foundations of the biology known before the 1980s by bringing out a new and puzzling type of infectious agent: prions.

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