A5094098996- Prion Diseases and Protein Misfolding
- Neurological diseases and metabolism
CIC bioGUNE
2024-2025
Abstract Prion diseases are a group of rapidly progressing neurodegenerative disorders caused by the misfolding endogenous prion protein (PrP C ) into pathogenic form Sc ). This process, despite being central event underlying these disorders, remains largely unknown at molecular level, precluding prediction new potential outbreaks or interspecies transmission incidents. In this work, we present method to generate bona fide recombinant prions de novo, allowing comprehensive analysis across...
Prion diseases, particularly sporadic cases, pose a challenge due to their complex nature and heterogeneity. The underlying mechanism of the spontaneous conversion from PrP C Sc , hallmark prion remains elusive. To shed light on this process involvement cofactors, we have developed an in vitro system that faithfully mimics misfolding using minimal components. By employing PMSA methodology introducing isoleucine residue at position 108 mouse PrP, successfully generated recombinant murine...