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Opa1 Overexpression Ameliorates the Phenotype of Two Mitochondrial Disease Mouse Models
Open accessAbstract
Increased levels of the mitochondria-shaping protein Opa1 improve respiratory chain efficiency and protect from tissue damage, suggesting that it could be an attractive target to counteract mitochondrial dysfunction. Here we show that Opa1 overexpression ameliorates two mouse models of defective mitochondrial bioenergetics. The offspring from crosses of a constitutive knockout for the structural complex I component Ndufs4 (Ndufs4(-/-)), and of a muscle-specific conditional knockout for the complex IV assembly factor Cox15 (Cox15(sm/sm)), with Opa1 transgenic (Opa1(tg)) mice showed improved motor skills and respiratory chain activities compared to the naive, non-Opa1-overexpressing, models. While the amelioration was modest in Ndufs4(-/-)::Opa1(tg) mice, correction of cristae ultrastructure and mitochondrial respiration, improvement of motor performance and prolongation of lifespan were remarkable in Cox15(sm/sm)::Opa1(tg) mice. Mechanistically, respiratory chain supercomplexes were increased in Cox15(sm/sm)::Opa1(tg) mice, and residual monomeric complex IV was stabilized. In conclusion, cristae shape amelioration by controlled Opa1 overexpression improves two mouse models of mitochondrial disease.
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OPA1 transgenic mice are protected from acute muscle loss induced by denervation and from chronic muscle loss in a model of myopathy caused by muscle-specific deletion of COX15.
“OPA1 transgenic mice are protected from acute muscle loss induced by denervation [194] as well as from chronic muscle loss in a model of myopathy caused by muscle-specific deletion of the mitochondrial subunit COX15 [195].”
From The connection between the dynamic remodeling of the mitochondrial network and the regulation of muscle mass (2021) · cites this paper as [195]
Rebalancing of mitochondrial dynamics rescues the phenotype of certain diseases associated with alterations of mitochondrial fusion or fission.
“Several reports have shown that rebalancing of mitochondrial dynamics rescues the phenotype of certain diseases associated with alterations of mitochondrial fusion or fission [119, 194, 195, 214]”
From The connection between the dynamic remodeling of the mitochondrial network and the regulation of muscle mass (2021) · cites this paper as [195]
Overexpression of Opa1 protects from denervation-induced muscle loss and ameliorates muscle function in a mouse model of mitochondrial myopathy.
“Conversely, overexpression of Opa1 protects from denervation-induced muscle loss and ameliorates muscle function in a mouse model of mitochondrial myopathy [9,10].”
From Inhibition of the Fission Machinery Mitigates OPA1 Impairment in Adult Skeletal Muscles (2019) · cites this paper as [9]
OPA1 overexpression protects mice from acute and chronic muscle atrophy by ameliorating mitochondrial cristae remodeling and mitochondrial dysfunction.
“Conversely, OPA1 overexpression protects mice from acute and chronic muscle atrophy by ameliorating mitochondrial cristae remodeling and mitochondrial dysfunction (87, 88).”
From From mitochondria to sarcopenia: role of 17β-estradiol and testosterone (2023) · cites this paper as [88]
OPA1 has important roles in regulating skeletal muscle mass and functions.
“Both transgenic and gene knockout approaches in mice have also been used to demonstrate the important roles of OPA1 in regulating skeletal muscle mass and functions23,44,45.”
From Disuse-associated loss of the protease Lonp1 in muscle impairs mitochondrial function and causes reduced skeletal muscle mass and strength (2022) · cites this paper as [44]
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Metadata
- Authors
- Civiletto, G., Varanita, T., Cerutti, R., Gorletta, T., Barbaro, S., Marchet, S., Lamperti, C., Viscomi, C., Scorrano, L., & Zeviani, M.
- Affiliations
- Fondazione IRCCS Istituto Neurologico Carlo Besta (IT)
- Veneto Institute of Molecular Medicine (IT)
- MRC Mitochondrial Biology Unit (GB)
- University of Padua (IT)
- Corresponding author
- Scorrano, Luca
- Zeviani, Massimo
- ORCID
- Venue
- Cell Metabolism
- Publisher
- Elsevier BV
- Published
- 2015-06
- Volume
- 21
- Issue
- 6
- Pages
- 845-854
- ISSN
- 1550-4131 (ISSN-L)
- PMC
- PMC4457891
- OpenAlex
- W2169501919
- Cited by
- 216
- References
- 50
- Citations by year
- 2026: 4 · 2025: 12 · 2024: 12 · 2023: 15 · 2022: 19 · 2021: 26 · 2020: 26 · 2019: 28 · 2018: 22 · 2017: 18 · 2016: 24 · 2015: 10
- FWCI
- 10.65
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Cell Metabolism (publishedVersion, cc-by-nc-nd)
- Europe PMC (PubMed Central) (publishedVersion, cc-by-nc-nd)
- PubMed Central (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- Archivio Istituzionale della Ricerca (Universita Degli Studi Di Milano) (submittedVersion, other-oa)
- Padua Research Archive (University of Padova) (submittedVersion, cc-by-nc-nd)
- Padua Research Archive (University of Padova) (submittedVersion, other-oa)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Autophagy in Disease and Therapy
- Keywords
- OPA1, mitochondrial disease, mitochondrial cristae, mitochondrial fusion, NDUFS4, complex I, cytochrome c oxidase, respiratory chain supercomplexes, mitochondrial bioenergetics, motor function, lifespan extension, transgenic mice, mitochondrial dysfunction
- MeSH terms
- Animals, Electron Transport Complex IV, Mitochondria, Oxygen Consumption, Gene Expression Regulation, Enzymologic, Mice, Knockout, GTP Phosphohydrolases, Mitochondrial Diseases, Electron Transport Complex I, Mice
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