Recent advances in the diagnosis and therapeutics for human prion diseases

Prion diseases, or transmissible spongiform encephalopathies, are fatal, neurodegenerative disorders associated with the accumulation of a misfolded infectious prion protein which is made by a posttranslational conformational change of the host-encoded cellular prion protein. A large number of studies to reveal the pathogenesis of prion diseases have been done using such experimental models as animals, cell cultures and cell-free systems over the past 30 years. The prion pathogenesis is still enigmatic, but current explosion of the knowledge about prion biology has led to the discovery of either more reliable diagnostic measurements or more beneficial therapeutic candidates. Here, the recent advances are reviewed in the diagnostics and the therapeutics for prion diseases.