Unexpected decrease of full-length prion protein in macaques inoculated with prion-contaminated blood products.

ABSTRACT: The presence of prion infectivity in the blood of patients affected by variant Creutzfeldt-Jakob disease (v-CJD), the human prion disease linked to the bovine spongiform encephalopathy (BSE), poses the risk of inter-human transmission of this fatal prion disease through transfusion. In the frame of various experiments, we have previously described that several cynomolgus macaques experimentally exposed to prion-contaminated blood products developed c-BSE/v-CJD, but the vast majority of them developed an unexpected, fatal disease phenotype focused on spinal cord involvement, which does not fulfill the classical diagnostic criteria of v-CJD. Here, we show that extensive analyses with current conventional techniques failed to detect any accumulation of abnormal prion protein (PrP^v-CJD

SUBMITTER: Jaffre N 

PROVIDER: S-EPMC10196267 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature