EXPERIMENTAL-MODELS REGARDING THE CAUSE O F SPONGIFORM ENCEPHALOPATHIES

Transmissible spongiform encephalopathies seem to contradict a dogma i n microbiology. There is now increasing evidence that the infectious a gents are proteins (prion proteins). These proteins seem to be able to catalyze conformational conversions of a host-encoded isoform. The al tered conformation induces intracellular accumulation and may lead to polymerization into fibrilles and amyloid rods. Catalytical interactio ns of infectious prion proteins and their cellular isoforms are depend ent on the primary structure. These considerations may be helpful to e valuate the risk of transmission of BSE to humans.