Prion Protein Peptide: Agents of Death for Neurons

The fundamental problem in addressing prion diseases, or the transmissible spongiform encephalopathies, is finding an explanation for the massive neuronal death that occurs. Although some understanding of the mechanism by which neuronal death occurs comes from studies with scrapie-infected mice, most of the insights regarding a possible mechanism have come from cell culture models in which a synthetic peptide (PrP106-126), based on the sequence of the prion protein, has been applied to neuronal cells. This review describes the details of the mechanism of toxicity of this peptide to cells.