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Cited by 1,002Mitochondrial Function and PathologyAutophagy in Disease and TherapyParkinson's Disease Mechanisms and Treatments

Mitochondrial fragmentation in neurodegeneration.

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Knott, Andrew B., Perkins, Guy, Schwarzenbacher, Robert, Bossy-Wetzel, Ella·2008·Nature Reviews Neuroscience
DOI 10.1038/nrn2417PMC PMC2711514
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Abstract

Mitochondria are remarkably dynamic organelles that migrate, divide and fuse. Cycles of mitochondrial fission and fusion ensure metabolite and mitochondrial DNA mixing and dictate organelle shape, number and bioenergetic functionality. There is mounting evidence that mitochondrial dysfunction is an early and causal event in neurodegeneration. Mutations in the mitochondrial fusion GTPases mitofusin 2 and optic atrophy 1, neurotoxins and oxidative stress all disrupt the cable-like morphology of functional mitochondria. This results in impaired bioenergetics and mitochondrial migration, and can trigger neurodegeneration. These findings suggest potential new treatment avenues for neurodegenerative diseases.

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  • Mitochondrial fragmentation is a significant factor in neurodegenerative diseases.

    “Mitochondrial fragmentation, a process characterized by the division of mitochondria into smaller, dysfunctional organelles, has emerged as a significant factor in neurodegenerative diseases (Knott et al., 2008).”

    From Aging promotes an increase in mitochondrial fragmentation in astrocytes (2024) · cites this paper as Knott et al., 2008

    The Abstract and Introduction link disrupted mitochondrial dynamics to neurodegeneration, supporting the claim that fragmentation is a significant factor. However, the Mitochondrial Dynamics and Bioenergetics section reports that some cells have fragmented mitochondria without bioenergetic defects, so the sentence overstates the claim that the resulting organelles are necessarily dysfunctional. (LLM, conf=0.86)

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Authors
Knott, A. B., Perkins, G., Schwarzenbacher, R., & Bossy-Wetzel, E.
Affiliations
  • University of Central Florida (US)
  • University of California San Diego (US)
  • University of Salzburg (AT)
ORCID
Perkins, Guy
Venue
Nature Reviews Neuroscience
Publisher
Springer Science and Business Media LLC
Published
2008-07
Volume
9
Issue
7
Pages
505-518
ISSN
  • 1471-003X (ISSN-L)
  • 1471-0048
DOI
10.1038/nrn2417
PMC
PMC2711514
OpenAlex
W2074052524
Cited by
1,002
References
22
Citations by year
2026: 30 · 2025: 42 · 2024: 41 · 2023: 43 · 2022: 56 · 2021: 51 · 2020: 70 · 2019: 56 · 2018: 66 · 2017: 54 · 2016: 68 · 2015: 44 · 2014: 56 · 2013: 72 · 2012: 66
FWCI
4.84
Type
journal article
Language
en
Open access
Licence
Locations
  • Nature reviews. Neuroscience (publishedVersion)
  • PubMed (publishedVersion)
  • PubMed Central (acceptedVersion)
  • Journal of International Crisis and Risk Communication Research (submittedVersion)
Funders
  • National Eye Institute (R01EY016164)
  • National Institute of Neurological Disorders and Stroke (R01NS047456, R01NS055193)
  • National Institute of Environmental Health Sciences (P42ES010337)
  • National Center for Research Resources (P41RR004050)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
  • Health Sciences › Medicine › Epidemiology › Autophagy in Disease and Therapy
  • Health Sciences › Medicine › Neurology › Parkinson's Disease Mechanisms and Treatments
Keywords
mitochondrial fragmentation, mitochondrial fission, mitochondrial fusion, neurodegeneration, mitochondrial dysfunction, mitofusin 2, optic atrophy 1, bioenergetics, oxidative stress, neurotoxins, neurodegenerative disease treatment
MeSH terms
Animals, Humans, Membrane Fusion, Membrane Proteins, Mitochondria, Models, Molecular, Neurons, Protein Conformation, Reactive Oxygen Species, Oxidative Stress, Neurodegenerative Diseases, Mitochondrial Proteins, Mitochondrial Membranes

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