Biochemical analysis of peripheral tissue involvement in transmissible spongiform encephalopathies

Abstract

Transmissible spongiform encephalopathies (TSE) are transmissible, neurodegenerative disorders affecting humans and animals. Although the etiological agent of TSE has yet to be conclusively identified, a unifying feature is the accumulation of an abnormal protease-resistant isomer (PrP^Sc) of the host encoded prion protein (PrP^C). PrP^C expression is required for the transmission and pathogenesis of TSE disease. Despite the ubiquitous organ distribution of PrP^C, TSE pathogenesis and infectivity is principally restricted to the central nervous and lymphoreticular systems. However, recent studies have highlighted the presence of low levels of PrP^Sc and TSE infectivity in some peripheral tissues including muscle and the possible transmission of variant Creutzfeldt Jakob disease through blood transfusion. This has raised concerns regarding the risk of iatrogenic transmission of TSE disease through surgical procedures and organ or blood donation.