Prion diseases are fatal spongiform neurodegenerative pathologies caused by the mutant protein PrPSc, that propagates by changing the conformation of its physiological counterpart PrPC. Since Gajdusek's first description of kuru in 1957 and Prusiner's 1982 prion hypothesis, evidence has accumulated on the pathogenesis of prion diseases. However, the role of PrPC was only described in 2016: long-term preservation of neuronal integrity through an interaction with a yet unidentified Schwann cell receptor...
- Prion diseases
- neurodegenerative pathologies
- mutant protein