Generation of human chronic wasting disease in transgenic mice.

ABSTRACT: Chronic wasting disease (CWD) is a cervid prion disease caused by the accumulation of an infectious misfolded conformer (PrP^Sc) of cellular prion protein (PrP^C). It has been spreading rapidly in North America and also found in Asia and Europe. Although bovine spongiform encephalopathy (i.e. mad cow disease) is the only animal prion disease known to be zoonotic, the transmissibility of CWD to humans remains uncertain. Here we report the generation of the first CWD-derived infectious human PrP^Sc by elk CWD PrP^Sc-seeded conversion of PrP^C in normal human brain homogenates using in vitro protein misfolding cyclic amplification (PMCA). Western blotting with human PrP selective antibody confirmed that the PMCA-generated protease-resistant Pr

SUBMITTER: Wang Z 

PROVIDER: S-EPMC8474769 | biostudies-literature | 2021 Sep

REPOSITORIES: biostudies-literature