Endoplasmic Reticulum Stress in Neurodegeneration

Jeroen J.M. Hoozemans; Wiep Scheper

doi:https://doi.org/10.1007/978-1-4020-9434-7_5

Endoplasmic Reticulum Stress in Neurodegeneration

Research output: Chapter in Book/Report/Conference proceeding › Chapter › Academic › peer-review

Abstract

Accumulation of misfolded proteins in the endoplasmic reticulum triggers a cellular stress response called the unfolded protein response (UPR) that protects the cell against the toxic buildup of misfolded proteins. Neurodegenerative disorders like Alzheimer’s disease, Parkinson’s disease, prion disease, Huntington’s disease, frontotemporal dementia, and amyotrophic lateral sclerosis are characterized by the accumulation and aggregation of misfolded proteins. In this chapter we will discuss the different levels of protein quality control systems in the endoplasmic reticulum. The role of these systems and especially the UPR will be reviewed in view of current data about the expression and role of the UPR markers in the pathology of neurodegenerative disorders.

Original language	English
Title of host publication	Protein Folding and Misfolding: Neurodegenerative Diseases
Editors	Judit Ovádi, Ferenc Orosz
Place of Publication	Dordrecht
Publisher	Springer Netherlands
Pages	111-132
Number of pages	22
ISBN (Print)	978-1-4020-9434-7
DOIs	https://doi.org/10.1007/978-1-4020-9434-7_5
Publication status	Published - 2009

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https://doi.org/10.1007/978-1-4020-9434-7_5

Cite this

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title = "Endoplasmic Reticulum Stress in Neurodegeneration",

abstract = "Accumulation of misfolded proteins in the endoplasmic reticulum triggers a cellular stress response called the unfolded protein response (UPR) that protects the cell against the toxic buildup of misfolded proteins. Neurodegenerative disorders like Alzheimer{\textquoteright}s disease, Parkinson{\textquoteright}s disease, prion disease, Huntington{\textquoteright}s disease, frontotemporal dementia, and amyotrophic lateral sclerosis are characterized by the accumulation and aggregation of misfolded proteins. In this chapter we will discuss the different levels of protein quality control systems in the endoplasmic reticulum. The role of these systems and especially the UPR will be reviewed in view of current data about the expression and role of the UPR markers in the pathology of neurodegenerative disorders.",

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AB - Accumulation of misfolded proteins in the endoplasmic reticulum triggers a cellular stress response called the unfolded protein response (UPR) that protects the cell against the toxic buildup of misfolded proteins. Neurodegenerative disorders like Alzheimer’s disease, Parkinson’s disease, prion disease, Huntington’s disease, frontotemporal dementia, and amyotrophic lateral sclerosis are characterized by the accumulation and aggregation of misfolded proteins. In this chapter we will discuss the different levels of protein quality control systems in the endoplasmic reticulum. The role of these systems and especially the UPR will be reviewed in view of current data about the expression and role of the UPR markers in the pathology of neurodegenerative disorders.

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