Protein Misfolding Cyclic Amplification of Infectious Prions.

Transmissible spongiform encephalopathies, or prion diseases, are a group of incurable disorders caused by the accumulation of an abnormally folded prion protein (PrPSc) in the brain. According to the "protein-only" hypothesis, PrPSc is the infectious agent able to propagate the disease by acting as a template for the conversion of the correctly folded prion protein (PrPC) into the pathological isoform. Recently, the mechanism of PrPC 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 process associated to other neurodegenerative diseases, including Alzheimer's disease, Parkinson's disease, and frontotemporal lobar degeneration.