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

Auteurs-es

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

Mots-clés :

priones, enfermedades raras, enfermedades priónicas

Résumé

Las enfermedades raras son aquellas patologías que afectan a una proporción muy reducida de la población (menos de 50 casos por cada 100 000 personas). Por esta razón, la investigación sobre sus causas y mecanismos, algo imprescindible para dar con una forma de tratarlas o prevenirlas, es insuficiente. Por ello, los pacientes denuncien la falta de cobertura del sistema sanitario y la discriminación social que supone padecer una de estas patologías. Entre las enfermedades raras, se encuentran las denominadas enfermedades priónicas o encefalopatías espongiformes transmisibles. A pesar de ser relativamente conocidas gracias a la crisis sanitaria que supuso el “mal de las vacas locas” a finales del siglo pasado, se conoce todavía relativamente poco sobre estas patologías que afectan tanto a animales como a humanos. En este monográfico se pretende dar a conocer la fascinante historia y la diversidad de las enfermedades priónicas, que sacudieron los cimientos de la biología conocida hasta los años 80 al poner en escena a un nuevo y desconcertante tipo de agente infeccioso: los priones.

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