Protein Misfolding Disorders: A Trip into the ER

Indexed in: Scopus, EBSCO.

Neurodegenerative disorders such as Amyotrophic lateral sclerosis (ALS), Alzheimer’s disease (AD), Parkinson’s disease (PD), Prion-related disorders (PrD) and Huntington’s disease (HD) share a ...
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Endoplasmic Reticulum Stress Response in Prion Diseases

Pp. 127-135 (9)

Abhisek Mukherjee and Claudio Soto


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.


Protein Misfolding Disorders Laboratory, George and Cynthia Mitchell Center for Neurodegenerative Diseases, Dept of Neurology, University of Texas Medical School at Houston.