Web of Science: 114 cites, Scopus: 123 cites, Google Scholar: cites,
Mitochondrial Quality Control in Neurodegenerative Diseases : Focus on Parkinson's Disease and Huntington's Disease
Franco Iborra, Sandra (Hospital Universitari Vall d'Hebron. Institut de Recerca)
Vila Bover, Miquel (Universitat Autònoma de Barcelona. Departament de Bioquímica i de Biologia Molecular)
Perier, Celine (Hospital Universitari Vall d'Hebron. Institut de Recerca)

Data: 2018
Resum: 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.
Ajuts: Ministerio de Economía y Competitividad SAF2016-77541-R
Ministerio de Economía y Competitividad RTC2014-2812-1
Ministerio de Economía y Competitividad PI13-01897
Drets: Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original. Creative Commons
Llengua: Anglès
Document: Article ; recerca ; Versió publicada
Matèria: Parkinson's disease ; Huntington's disease ; Mitochondrial quality control ; Mitochondrial dysfunction ; Neurodegeneration
Publicat a: Frontiers in Neuroscience, Vol. 12 (May 2018) , art. 342, ISSN 1662-453X

DOI: 10.3389/fnins.2018.00342
PMID: 29875626

25 p, 2.6 MB

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 Registre creat el 2018-07-30, darrera modificació el 2022-03-30

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