Chapter 11

Endoplasmic Reticulum Stress Response in Prion Diseases

Abhisek Mukherjee and Claudio Soto

Abstract

Prion diseases are infectious neurodegenerative diseases associated with the brain accumulation of the misfolded prion protein. Despite substantial knowledge of the mechanism of infection and disease transmission, little is known about the molecular pathways involved in neurodegeneration. Recent findings implicate endoplasmic reticulum (ER) stress as a key event in brain dysfunction. The available evidence indicates that accumulation of misfolded prion protein induces ER stress, followed by the activation of the unfolded protein response. Chronic stress produced by the sustained formation of misfolded proteins lead to neuronal apoptosis and synaptic alterations. In this article we discuss the role of ER stress in prion neurodegeneration and the signaling pathways implicated.

Total Pages: 127-135 (9)

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