The Prion Basis of Progressive Neurodegenerative Disorders.

The discovery of proteinaceous infectious agents by Prusiner in 1982 was sensational. All previously known pathogens contained nucleic acids, the code of life, that enabled them to reproduce. In contrast, the proteinaceous agents of disease, called prion proteins (PrP), lacked nucleic acids and propagated by binding to the functional, endogenous form of cellular prion protein (referred to as PrP) and altering its conformation to produce the infectious disease-causing misfolded protein (referred to as PrP).