The strain-encoded relationship between PrP replication, stability and processing in neurons is predictive of the incubation period of disease.

ABSTRACT: Prion strains are characterized by differences in the outcome of disease, most notably incubation period and neuropathological features. While it is established that the disease specific isoform of the prion protein, PrP(Sc), is an essential component of the infectious agent, the strain-specific relationship between PrP(Sc) properties and the biological features of the resulting disease is not clear. To investigate this relationship, we examined the amplification efficiency and conformational stability of PrP(Sc) from eight hamster-adapted prion strains and compared it to the resulting incubation period of disease and processing of PrP(Sc) in neurons and glia. We found that short incubation period strains were characterized by more efficient PrP(Sc) amplification and higher PrP(Sc) conform

SUBMITTER: Ayers JI 

PROVIDER: S-EPMC3060105 | biostudies-literature | 2011 Mar

REPOSITORIES: biostudies-literature