ABSTRACT
Although transmissible spongiform encephalopathies (TSEs) are diseases without a unique etiology or clinical profile, the characteristics of brain damage are similar. Extensive spongiform degeneration, a large extent of neuronal loss, synaptic alterations, atypical brain inflammation, and accumulation of protein aggregates are typical features of TSE [Castilla et al. 2004]. This chapter describes the features of brain degeneration, the putative relationship of the prion protein and its structural conversion with brain damage, and the signaling pathways involved in TSE neurodegeneration.
