Prion diseases are caused by a conformational conversion of the cellular prion protein (PrPC) to a pathological conformer (PrPSc). The “prion-only” hypothesis suggests that PrPSc is the infectious agent that propagates the disease acting as a template for theconversion of PrPC. In 2001, we developed a novel in vitro technique, called Protein misfolding cyclic ampli fi cation (PMCA), which mimics this pathological process in an accelerated way. Thereby, minimal amount of PrPSc can be ampli fi ed to several millions fold, providing an important tool for diagnosis and investigation of prion biology, and the molecular mechanism of prion conversion. PMCA also offers a great platform for the study and ampli fi cation of the protein misfolding process associated with other neurodegenerative disorders, such as Alzheimer’s and Parkinson’s diseases.
- Prion decontamination procedures
- Prion diseases
- Prion transmission
- Protein misfolding cyclic amplification
- Transmissible spongiform encephalopathies
ASJC Scopus subject areas