Purification and Characterization of the Prion of Creutzfeldt-Jakob Disease

摘要

Creutzfeldt-Jakob disease (CJD) is one of several central nervous system diseases of animal and human recognized as transmissible spongiform encephalopathies. While these disease are clearly associated with transmissible agents which replicate to high titer in the affected host, the biochemical properties of the agents have proven to be elusive and conflicting. It is widely believed that the so-called Unconventional Viruses share a common set of properties, and that insight into one will provide the conceptual framework needed to define the group as a whole. This chapter describes the biology and biochemistry of the CJD agent and compares these properties with those of the scrapie prion. The data support the argument that both are members of a new group of infectious agents called prions.