Characterisation of the pathological prion protein for cases of transmissible spongiform encephalopathies in the Basque Country and study of their epidemiology and pathogenesis

  • Financing: Department of Economic Development and Competitiveness. Basque Government.
  • Head researcher: Ramón Juste
  • Implementation: 2015

The overall objective of this project is the study of the encephalopathies caused by prions.

The specific objectives are:

1. To apply the protocol for the molecular characterisation of the prion protein to cases in the Basque Country.
2. To contribute to the characterisation of clinical and histopathologic forms.
3. To study possible molecular and epidemiological relationships in human and animal cases.
4. To improve the sensitivity and specificity of diagnostic methods.
5. To study the sensitivity of different molecular subtypes of pathogenic prion protein to degradation with proteases and their relationship with the incubation time in transgenic mouse models.
6. Generation of models for spontaneous prion disease in mice.