Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease
In recent years, several important advances have been made in our understanding of the pathways that lead to cell dysfunction and death in Parkinson's disease (PD) and Huntington's disease (HD). Despite distinct clinical and pathological features, these two neurodegenerative diseases share...
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doaj-9f2a0918d5074aceb057732aeb00bc6b2020-11-25T01:09:22ZengFrontiers Media S.A.Frontiers in Neuroscience1662-453X2018-05-011210.3389/fnins.2018.00342365850Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's DiseaseSandra Franco-Iborra0Miquel Vila1Miquel Vila2Miquel Vila3Celine Perier4Vall d'Hebron Research Institute (VHIR)-Center for Networked Biomedical Research in Neurodegenerative Diseases (CIBERNED), Barcelona, SpainVall d'Hebron Research Institute (VHIR)-Center for Networked Biomedical Research in Neurodegenerative Diseases (CIBERNED), Barcelona, SpainCatalan Institution for Research and Advanced Studies, Barcelona, SpainDepartment of Biochemistry and Molecular Biology, Autonomous University of Barcelona, Barcelona, SpainVall d'Hebron Research Institute (VHIR)-Center for Networked Biomedical Research in Neurodegenerative Diseases (CIBERNED), Barcelona, SpainIn recent years, several important advances have been made in our understanding of the pathways that lead to cell dysfunction and death in Parkinson's disease (PD) and Huntington's disease (HD). Despite distinct clinical and pathological features, these two neurodegenerative diseases share critical processes, such as the presence of misfolded and/or aggregated proteins, oxidative stress, and mitochondrial anomalies. Even though the mitochondria are commonly regarded as the “powerhouses” of the cell, they are involved in a multitude of cellular events such as heme metabolism, calcium homeostasis, and apoptosis. Disruption of mitochondrial homeostasis and subsequent mitochondrial dysfunction play a key role in the pathophysiology of neurodegenerative diseases, further highlighting the importance of these organelles, especially in neurons. The maintenance of mitochondrial integrity through different surveillance mechanisms is thus critical for neuron survival. Mitochondria display a wide range of quality control mechanisms, from the molecular to the organellar level. Interestingly, many of these lines of defense have been found to be altered in neurodegenerative diseases such as PD and HD. Current knowledge and further elucidation of the novel pathways that protect the cell through mitochondrial quality control may offer unique opportunities for disease therapy in situations where ongoing mitochondrial damage occurs. In this review, we discuss the involvement of mitochondrial dysfunction in neurodegeneration with a special focus on the recent findings regarding mitochondrial quality control pathways, beyond the classical effects of increased production of reactive oxygen species (ROS) and bioenergetic alterations. We also discuss how disturbances in these processes underlie the pathophysiology of neurodegenerative disorders such as PD and HD.https://www.frontiersin.org/article/10.3389/fnins.2018.00342/fullParkinson's diseaseHuntington's diseasemitochondrial quality controlmitochondrial dysfunctionneurodegeneration |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Sandra Franco-Iborra Miquel Vila Miquel Vila Miquel Vila Celine Perier |
spellingShingle |
Sandra Franco-Iborra Miquel Vila Miquel Vila Miquel Vila Celine Perier Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease Frontiers in Neuroscience Parkinson's disease Huntington's disease mitochondrial quality control mitochondrial dysfunction neurodegeneration |
author_facet |
Sandra Franco-Iborra Miquel Vila Miquel Vila Miquel Vila Celine Perier |
author_sort |
Sandra Franco-Iborra |
title |
Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease |
title_short |
Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease |
title_full |
Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease |
title_fullStr |
Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease |
title_full_unstemmed |
Mitochondrial Quality Control in Neurodegenerative Diseases: Focus on Parkinson's Disease and Huntington's Disease |
title_sort |
mitochondrial quality control in neurodegenerative diseases: focus on parkinson's disease and huntington's disease |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Neuroscience |
issn |
1662-453X |
publishDate |
2018-05-01 |
description |
In recent years, several important advances have been made in our understanding of the pathways that lead to cell dysfunction and death in Parkinson's disease (PD) and Huntington's disease (HD). Despite distinct clinical and pathological features, these two neurodegenerative diseases share critical processes, such as the presence of misfolded and/or aggregated proteins, oxidative stress, and mitochondrial anomalies. Even though the mitochondria are commonly regarded as the “powerhouses” of the cell, they are involved in a multitude of cellular events such as heme metabolism, calcium homeostasis, and apoptosis. Disruption of mitochondrial homeostasis and subsequent mitochondrial dysfunction play a key role in the pathophysiology of neurodegenerative diseases, further highlighting the importance of these organelles, especially in neurons. The maintenance of mitochondrial integrity through different surveillance mechanisms is thus critical for neuron survival. Mitochondria display a wide range of quality control mechanisms, from the molecular to the organellar level. Interestingly, many of these lines of defense have been found to be altered in neurodegenerative diseases such as PD and HD. Current knowledge and further elucidation of the novel pathways that protect the cell through mitochondrial quality control may offer unique opportunities for disease therapy in situations where ongoing mitochondrial damage occurs. In this review, we discuss the involvement of mitochondrial dysfunction in neurodegeneration with a special focus on the recent findings regarding mitochondrial quality control pathways, beyond the classical effects of increased production of reactive oxygen species (ROS) and bioenergetic alterations. We also discuss how disturbances in these processes underlie the pathophysiology of neurodegenerative disorders such as PD and HD. |
topic |
Parkinson's disease Huntington's disease mitochondrial quality control mitochondrial dysfunction neurodegeneration |
url |
https://www.frontiersin.org/article/10.3389/fnins.2018.00342/full |
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