ABSTRACT
Prion disease is a rare, progressive, fatal, and transmissible neurodegenerative disease that is characterized by spongiform encephalopathy (TSE) in the brain of certain mammals including humans. The interaction between normal prion protein PrPc and infectious PrPsc causes conversion of PrPc to PrPsc, and this process is repeated leading to accumulation of PrPsc in the secondary lymphoid organs from where transported by peripheral nerves to the brain where they continue to replicate and cause inflammation, which contributes to the progression of prion. Some studies suggest that increased oxidative stress and chronic inflammation are involved in the pathogenesis of this disease. Therefore, reducing these cellular defects may help in the management of prion disease. This chapter describes incidence, conversion of PrPc to PrPsc, pathological changes in the brain, role of microRNAs and polymorphisms in pathogenesis, and influence of Nrf2 in reducing oxidative stress and inflammation. In addition, it proposes that in order to simultaneously reduce oxidative stress and inflammation, it is essential to enhance the levels of antioxidant enzymes by activating the Nrf2 pathway, and dietary and endogenous antioxidants by supplementation. A mixture of micronutrients that would achieve the above goal is proposed. This mixture may improve the management of prion diseases.
