Transmissible spongiform encephalopathies: a family of etiologically complex diseases—a review

The upsurge of ‘mad cow disease’ with its human implications has raised the problem of the etiological mechanisms and the similarities or differences underlying the family of transmissible spongiform encephalopathies. Structural properties of prions are reviewed in connection with their natural distribution and functions, factors of transmissibility and mechanisms of pathogenicity. Polymorphism is examined in relation to disease phenotype variants. The role of oxidative factors is emphasized, while raising complexity about the role of copper ions. Further investigation directions are suggested.