Therapeutical approaches for prion diseases and characterization of recombinant prion strains from different perspectives

  1. López Lorenzo, Nuria
Dirigida por:
  1. Jesús Rodríguez Requena Director

Universidad de defensa: Universidade de Santiago de Compostela

Año de defensa: 2023

  1. Joaquín Castilla Castrillón Presidente/a
  2. Carmen Rivas Vázquez Secretaria
  3. Ilaria Vani Vocal
  1. Departamento de Psiquiatría, Radiología, Salud Pública, Enfermería y Medicina

Tipo: Tesis


Prion diseases are rare and fatal neurodegenerative disorders for which there is no cure or treatment. They are caused by infectious proteins that result from the misfolding of the cellular prion protein, PrPC, into its pathogenic form, PrPSc. Furthermore, PrPSc can appear as conformational variants called strains that exhibit distinct phenotypes and could present resistance to anti-prion compounds. This thesis aims to assess three different therapeutic candidates in a fast murine model of prion disease to tackle these disorders from different aspects of their biology. Additionally, due to the role of prion strains in drug resistance and their biochemical and structural interest, diverse recombinant prion strains were characterized in this thesis using biological, biochemical, and biophysical methods.