The Endoplasmic Reticulum Stress/Unfolded Protein Response and Their Contributions to Parkinson’s Disease Physiopathology

Parkinson’s disease (PD) is a multifactorial age-related movement disorder in which defects of both mitochondria and the endoplasmic reticulum (ER) have been reported. The unfolded protein response (UPR) has emerged as a key cellular dysfunction associated with the etiology of the disease. The UPR i...

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Published in:Cells
Main Authors: Cristine Alves da Costa, Wejdane El Manaa, Eric Duplan, Frédéric Checler
Format: Article
Language:English
Published: MDPI AG 2020-11-01
Subjects:
Online Access:https://www.mdpi.com/2073-4409/9/11/2495
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author Cristine Alves da Costa
Wejdane El Manaa
Eric Duplan
Frédéric Checler
author_facet Cristine Alves da Costa
Wejdane El Manaa
Eric Duplan
Frédéric Checler
author_sort Cristine Alves da Costa
collection DOAJ
container_title Cells
description Parkinson’s disease (PD) is a multifactorial age-related movement disorder in which defects of both mitochondria and the endoplasmic reticulum (ER) have been reported. The unfolded protein response (UPR) has emerged as a key cellular dysfunction associated with the etiology of the disease. The UPR involves a coordinated response initiated in the endoplasmic reticulum that grants the correct folding of proteins. This review gives insights on the ER and its functioning; the UPR signaling cascades; and the link between ER stress, UPR activation, and physiopathology of PD. Thus, <i>post-mortem</i> studies and data obtained by either <i>in vitro</i> and <i>in vivo</i> pharmacological approaches or by genetic modulation of PD causative genes are described. Further, we discuss the relevance and impact of the UPR to sporadic and genetic PD pathology.
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spelling doaj-art-be13399caafa40a2bab63fc0c07674cd2025-08-19T22:30:19ZengMDPI AGCells2073-44092020-11-01911249510.3390/cells9112495The Endoplasmic Reticulum Stress/Unfolded Protein Response and Their Contributions to Parkinson’s Disease PhysiopathologyCristine Alves da Costa0Wejdane El Manaa1Eric Duplan2Frédéric Checler3INSERM, CNRS, IPMC, Team Labeled “Laboratory of Excellence (LABEX) Distalz”, Université Côte d’Azur, 660 Route des Lucioles, Sophia-Antipolis, 06560 Valbonne, FranceINSERM, CNRS, IPMC, Team Labeled “Laboratory of Excellence (LABEX) Distalz”, Université Côte d’Azur, 660 Route des Lucioles, Sophia-Antipolis, 06560 Valbonne, FranceINSERM, CNRS, IPMC, Team Labeled “Laboratory of Excellence (LABEX) Distalz”, Université Côte d’Azur, 660 Route des Lucioles, Sophia-Antipolis, 06560 Valbonne, FranceINSERM, CNRS, IPMC, Team Labeled “Laboratory of Excellence (LABEX) Distalz”, Université Côte d’Azur, 660 Route des Lucioles, Sophia-Antipolis, 06560 Valbonne, FranceParkinson’s disease (PD) is a multifactorial age-related movement disorder in which defects of both mitochondria and the endoplasmic reticulum (ER) have been reported. The unfolded protein response (UPR) has emerged as a key cellular dysfunction associated with the etiology of the disease. The UPR involves a coordinated response initiated in the endoplasmic reticulum that grants the correct folding of proteins. This review gives insights on the ER and its functioning; the UPR signaling cascades; and the link between ER stress, UPR activation, and physiopathology of PD. Thus, <i>post-mortem</i> studies and data obtained by either <i>in vitro</i> and <i>in vivo</i> pharmacological approaches or by genetic modulation of PD causative genes are described. Further, we discuss the relevance and impact of the UPR to sporadic and genetic PD pathology.https://www.mdpi.com/2073-4409/9/11/2495Parkinson’s diseaseunfolded protein responsereticulum endoplasmicgenetics
spellingShingle Cristine Alves da Costa
Wejdane El Manaa
Eric Duplan
Frédéric Checler
The Endoplasmic Reticulum Stress/Unfolded Protein Response and Their Contributions to Parkinson’s Disease Physiopathology
Parkinson’s disease
unfolded protein response
reticulum endoplasmic
genetics
title The Endoplasmic Reticulum Stress/Unfolded Protein Response and Their Contributions to Parkinson’s Disease Physiopathology
title_full The Endoplasmic Reticulum Stress/Unfolded Protein Response and Their Contributions to Parkinson’s Disease Physiopathology
title_fullStr The Endoplasmic Reticulum Stress/Unfolded Protein Response and Their Contributions to Parkinson’s Disease Physiopathology
title_full_unstemmed The Endoplasmic Reticulum Stress/Unfolded Protein Response and Their Contributions to Parkinson’s Disease Physiopathology
title_short The Endoplasmic Reticulum Stress/Unfolded Protein Response and Their Contributions to Parkinson’s Disease Physiopathology
title_sort endoplasmic reticulum stress unfolded protein response and their contributions to parkinson s disease physiopathology
topic Parkinson’s disease
unfolded protein response
reticulum endoplasmic
genetics
url https://www.mdpi.com/2073-4409/9/11/2495
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