Protein Misfolding Cyclic Amplification of Infectious Prions

Fabio Moda

doi:10.1016/bs.pmbts.2017.06.016

Protein Misfolding Cyclic Amplification of Infectious Prions

Fabio Moda

Fondazione IRCCS Istituto Neurologico "C. Besta"

Research output: Chapter in Book/Report/Conference proceeding › Chapter

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
Title of host publication	Progress in Molecular Biology and Translational Science
Pages	361-374
Number of pages	14
Volume	150
DOIs	https://doi.org/10.1016/bs.pmbts.2017.06.016
Publication status	Published - Jan 1 2017

Keywords

Aggregation
Neurodegeneration
PMCA
Prion diseases
Prion-like

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10.1016/bs.pmbts.2017.06.016

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AB - © 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.

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Protein Misfolding Cyclic Amplification of Infectious Prions

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Keywords

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