Region-specific protein misfolding cyclic amplification reproduces brain tropism of prion strains.

ABSTRACT: Human prion diseases such as Creutzfeldt-Jakob disease are transmissible brain proteinopathies, characterized by the accumulation of a misfolded isoform of the host cellular prion protein (PrP) in the brain. According to the prion model, prions are defined as proteinaceous infectious particles composed solely of this abnormal isoform of PrP (PrP^Sc). Even in the absence of genetic material, various prion strains can be propagated in experimental models. They can be distinguished by the pattern of disease they produce and especially by the localization of PrP^Sc deposits within the brain and the spongiform lesions they induce. The mechanisms involved in this strain-specific targeting of distinct brain regions still are a fundamental, unresolved question in prion research

SUBMITTER: Privat N 

PROVIDER: S-EPMC5633130 | biostudies-literature | 2017 Oct

REPOSITORIES: biostudies-literature