Modeling of human prions and prion diseases in vitro and in vivo

Although prion diseases are rare, affecting about one person per million per year worldwide, they have recently received remarkable attention because of the epidemic of the newly recognized bovine spongiform encephalopathy (BSE) and the causative association of BSE with human variant Creutzfeldt–Jakob disease (vCJD). At present, prion diseases are incurable. Here, we review current strategies in the modeling of human prion propagation and human prion diseases in vitro and in vivo. Modeling of prion diseases has contributed and will continue to contribute to the understanding of the pathogenesis as well as of the therapeutics of these diseases, but crucial experiments remain elusive.