Oxidative Stress and Mitochondrial Dysfunction in Neurodegeneration of Transmissible Spongiform Encephalopathies (TSEs)

Chapter · December 2005with13 Reads
DOI: 10.1007/0-387-23923-5_8
In book: Neurodegeneration and Prion Disease, pp.195-216


    Transmissible spongiform encephalopathies (TSEs) or prion diseases are neurodegenerative disorders that are invariably fatal in humans and animals. An important component of the infectious agent is a glyco-protein, termed PrPSc, which is derived from a normal cellular protein, termed PrPc. The pathogenic mechanisms of TSEs are not clear, but several factors such as oxidative stress and mitochondrial dysfunction have been reported to be involved. In the current review, we will present data that supports a role for oxidative stress and mitochondrial dysfunction in the induction of these diseases. We will discuss the pathways whereby oxidative stress and mitochondrial dysfunction could lead to neuronal damage and the clinical manifestations of TSE diseases.