Mitochondrial fusion protects against neurodegeneration in the cerebellum
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Mutations in the mitochondrial fusion gene Mfn2 cause the human neurodegenerative disease Charcot-Marie-Tooth type 2A. However, the cellular basis underlying this relationship is poorly understood. By removing Mfn2 from the cerebellum, we established a model for neurodegeneration caused by loss of mitochondrial fusion. During development and after maturity, Purkinje cells require Mfn2 but not Mfn1 for dendritic outgrowth, spine formation, and cell survival. In vivo, cell culture, and electron microscopy studies indicate that mutant Purkinje cells have aberrant mitochondrial distribution, ultrastructure, and electron transport chain activity. In fibroblasts lacking mitochondrial fusion, the majority of mitochondria lack mitochondrial DNA nucleoids. This deficiency provides a molecular mechanism for the dependence of respiratory activity on mitochondrial fusion. Our results show that exchange of mitochondrial contents is important for mitochondrial function as well as organelle distribution in neurons and have important implications for understanding the mechanisms of neurodegeneration due to perturbations in mitochondrial fusion.
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Mfn1, Mfn2 and Opa1 have roles in mtDNA maintenance in mammalian cells.
“The results of subsequent studies in mammalian cells agreed with these findings and confirmed the roles of Mfn1, Mfn2 and Opa1 in mtDNA maintenance (Chen et al., 2007).”
From New insights into the role of mitochondria in aging: mitochondrial dynamics and more (2010) · cites this paper as Chen et al., 2007
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- Authors
- Chen, H., McCaffery, J. M., & Chan, D. C.
- Affiliations
- California Institute of Technology (US)
- Johns Hopkins University (US)
- Corresponding author
- Chan, David C.
- Venue
- Cell
- Publisher
- Elsevier BV
- Published
- 2007-08
- Volume
- 130
- Issue
- 3
- Pages
- 548-562
- ISSN
- 0092-8674 (ISSN-L)
- OpenAlex
- W2024595059
- Cited by
- 924
- References
- 42
- Citations by year
- 2026: 27 · 2025: 34 · 2024: 34 · 2023: 45 · 2022: 43 · 2021: 54 · 2020: 57 · 2019: 52 · 2018: 69 · 2017: 53 · 2016: 47 · 2015: 40 · 2014: 44 · 2013: 48 · 2012: 92
- FWCI
- 18.30
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- www.elsevier.com/tdm/userlicense/1.0/ (tdm)
- www.elsevier.com/legal/tdmrep-license (tdm)
- www.elsevier.com/open-access/userlicense/1.0/ (vor, 2213 d)
- doi.org/10.15223/policy-017 (stm-asf)
- doi.org/10.15223/policy-037 (stm-asf)
- doi.org/10.15223/policy-012 (stm-asf)
- doi.org/10.15223/policy-029 (stm-asf)
- doi.org/10.15223/policy-004 (stm-asf)
- Locations
- Cell (publishedVersion)
- PubMed (publishedVersion)
- CaltechAUTHORS (California Institute of Technology) (submittedVersion)
- Funders
- United Mitochondrial Disease Foundation
- National Institutes of Health
- National Institute of General Medical Sciences (R01GM062967, GM062967)
- National Center for Research Resources (S10RR021023, S10RR019409, S10RR023454, S10RR022588)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Health Sciences › Medicine › Epidemiology › Autophagy in Disease and Therapy
- Keywords
- mitochondrial fusion, mitofusin 2, Mfn1, neurodegeneration, cerebellum, Purkinje cells, Charcot-Marie-Tooth disease type 2A, mitochondrial distribution, electron transport chain, dendritic outgrowth, spine formation
- MeSH terms
- Animals, Cerebellum, Intracellular Membranes, Membrane Fusion, Mice, Inbred C57BL, Mitochondria, Purkinje Cells, Mice, Knockout, Neurodegenerative Diseases, GTP Phosphohydrolases, Mice
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