Evidence for preexisting prion substrain diversity in a biologically cloned prion strain.

ABSTRACT: Prion diseases are a group of inevitably fatal neurodegenerative disorders affecting numerous mammalian species, including Sapiens. Prions are composed of PrPSc, the disease specific conformation of the host encoded prion protein. Prion strains are operationally defined as a heritable phenotype of disease under controlled transmission conditions. Treatment of rodents with anti-prion drugs results in the emergence of drug-resistant prion strains and suggest that prion strains are comprised of a dominant strain and substrains. While much experimental evidence is consistent with this hypothesis, direct observation of substrains has not been observed. Here we show that replication of the dominant strain is required for suppression of a substrain. Based on this observation we reasoned that sele

SUBMITTER: Gunnels T 

PROVIDER: S-EPMC10503715 | biostudies-literature | 2023 Sep

REPOSITORIES: biostudies-literature