Cited by 388Mitochondrial Function and PathologyParkinson's Disease Mechanisms and TreatmentsAlzheimer's disease research and treatments

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The Role of Mitochondria in Neurodegenerative Diseases: the Lesson from Alzheimer’s Disease and Parkinson’s Disease

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Monzio Compagnoni, Giacomo, Di Fonzo, Alessio, Corti, Stefania, Comi, Giacomo P., Bresolin, Nereo, Masliah, Eliezer·2020·Molecular Neurobiology

DOI 10.1007/s12035-020-01926-1PMC PMC9047992

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Abstract

Although the pathogenesis of neurodegenerative diseases is still widely unclear, various mechanisms have been proposed and several pieces of evidence are supportive for an important role of mitochondrial dysfunction. The present review provides a comprehensive and up-to-date overview about the role of mitochondria in the two most common neurodegenerative disorders: Alzheimer's disease (AD) and Parkinson's disease (PD). Mitochondrial involvement in AD is supported by clinical features like reduced glucose and oxygen brain metabolism and by numerous microscopic and molecular findings, including altered mitochondrial morphology, impaired respiratory chain function, and altered mitochondrial DNA. Furthermore, amyloid pathology and mitochondrial dysfunction seem to be bi-directionally correlated. Mitochondria have an even more remarkable role in PD. Several hints show that respiratory chain activity, in particular complex I, is impaired in the disease. Mitochondrial DNA alterations, involving deletions, point mutations, depletion, and altered maintenance, have been described. Mutations in genes directly implicated in mitochondrial functioning (like Parkin and PINK1) are responsible for rare genetic forms of the disease. A close connection between alpha-synuclein accumulation and mitochondrial dysfunction has been observed. Finally, mitochondria are involved also in atypical parkinsonisms, in particular multiple system atrophy. The available knowledge is still not sufficient to clearly state whether mitochondrial dysfunction plays a primary role in the very initial stages of these diseases or is secondary to other phenomena. However, the presented data strongly support the hypothesis that whatever the initial cause of neurodegeneration is, mitochondrial impairment has a critical role in maintaining and fostering the neurodegenerative process.

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Authors: Monzio Compagnoni, G., Di Fonzo, A., Corti, S., Comi, G. P., Bresolin, N., & Masliah, E.
Affiliations: University of Milan (IT)
National Institutes of Health (US)
ORCID: Monzio Compagnoni, Giacomo
Corti, Stefania
Comi, Giacomo P.
Bresolin, Nereo
Masliah, Eliezer
Venue: Molecular Neurobiology
Publisher: Springer Science and Business Media LLC
Published: 2020-05-22
Volume: 57
Issue: 7
Pages: 2959-2980
ISSN: 0893-7648 (ISSN-L)
1559-1182
DOI: 10.1007/s12035-020-01926-1
PMC: PMC9047992
OpenAlex: W3027900078
Cited by: 378
References: 309
Citations by year: 2026: 44 · 2025: 71 · 2024: 96 · 2023: 61 · 2022: 57 · 2021: 38 · 2020: 11
FWCI: 4.00
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: www.springer.com/tdm (tdm)
www.springer.com/tdm (vor)
Locations: Molecular Neurobiology (publishedVersion)
PubMed (publishedVersion)
PubMed Central (acceptedVersion)
Archivio Istituzionale della Ricerca (Universita Degli Studi Di Milano) (submittedVersion, other-oa)
Funders: Fresco Institute for Parkinson's and Movement Disorders (not available)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
Health Sciences › Medicine › Clinical Neurology › Parkinson's Disease Mechanisms and Treatments
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Keywords: mitochondria, mitochondrial dysfunction, neurodegenerative diseases, Alzheimer's disease, Parkinson's disease, mitochondrial DNA, electron transport chain, complex I, Parkin, PINK1, alpha-synuclein, amyloid pathology, multiple system atrophy, atypical parkinsonism, brain metabolism
MeSH terms: Alzheimer Disease, Animals, Brain, DNA, Mitochondrial, Humans, Mitochondria, Parkinson Disease, Electron Transport Complex I

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The Role of Mitochondria in Neurodegenerative Diseases: the Lesson from Alzheimer’s Disease and Parkinson’s Disease

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