Abstract
© 2017 Elsevier Inc. Transmissible spongiform encephalopathies, or prion diseases, are a group of incurable disorders caused by the accumulation of an abnormally folded prion protein (PrP Sc ) in the brain. According to the “protein-only” hypothesis, PrP Sc is the infectious agent able to propagate the disease by acting as a template for the conversion of the correctly folded prion protein (PrP C ) into the pathological isoform. Recently, the mechanism of PrP C conversion has been mimicked in vitro using an innovative technique named protein misfolding cyclic amplification (PMCA). This technology represents a great tool for studying diverse aspects of prion biology in the field of basic research and diagnosis. Moreover, PMCA can be expanded for the study of the misfolding proces s associated to other neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, and frontotemporal lobar degeneration.
Original language | English |
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Title of host publication | Progress in Molecular Biology and Translational Science |
Pages | 361-374 |
Number of pages | 14 |
Volume | 150 |
DOIs | |
Publication status | Published - Jan 1 2017 |
Keywords
- Aggregation
- Neurodegeneration
- PMCA
- Prion diseases
- Prion-like