Increased ROS generation and SOD activity in heteroplasmic tissues of transmitochondrial mice with A3243G mitochondrial DNA mutation
Open accessAbstract
The mitochondrial A3243G tRNALeu(UUR) mutation associated with a variety of mitochondrial disorders results in a severe respiratory deficiency, an increase in reactive oxygen species (ROS) production and activities of anti-oxidative enzyme in vitro. However, the phenotypic implications of this mutation have not been described in vivo. Here, mitochondria carrying A3243G transition from the peripheral blood of diabetes mellitus patients were microinjected into zygotes. Influence of this mutation on mitochondrial respiratory enzyme activities, ROS generation, and anti-oxidative enzyme activities in the heteroplasmic tissues of transmitochondrial mice was evaluated. The chimeric mice exhibited a subtle impaired oxidative phosphorylation, reduced activity of complex I/IV, increased activity of superoxide dismutase, and in turn, enhanced ROS generation. Our results suggest that mitochondrial A3243G mutation may be responsible for the high ROS production in vivo. Increased generation of ROS caused by mtDNA mutation may also play a role in the pathogenesis of the A3243G mutation-associated diseases.
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Injection of m.3243A N G mutated mitochondria into C57BL/6J mouse zygotes led to respiratory dysfunction and enhanced ROS production in heteroplasmic tissues.
“and the injection of m.3243A N G mutated mitochondria into C57BL/6J mouse zygotes led to respiratory dysfunction and enhanced ROS production in heteroplasmic tissues [175].”
From Mitochondrial DNA mutations and human disease (2010) · cites this paper as [175]
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- Authors
- Li, J., Zhou, K., Meng, X., Wu, Q., Li, S., Liu, Y., & Wang, J.
- Affiliations
- Capital Medical University (CN)
- Guangdong Medical College (CN)
- Venue
- Genetics and Molecular Research
- Publisher
- Genetics and Molecular Research
- Published
- 2008
- Volume
- 7
- Issue
- 4
- Pages
- 1054-1062
- ISSN
- 1676-5680 (ISSN-L)
- OpenAlex
- W2108131056
- Cited by
- 22
- References
- 29
- Citations by year
- 2025: 1 · 2024: 1 · 2021: 4 · 2020: 2 · 2019: 1 · 2018: 1 · 2015: 2 · 2013: 1 · 2012: 5
- FWCI
- 0.55
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Genetics and Molecular Research (publishedVersion)
- PubMed (publishedVersion)
- Funders
- University of Southern California
- Chinese Academy of Medical Sciences
- Peking University
- Peking Union Medical College
- 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 › Molecular Biology › Redox biology and oxidative stress
- Keywords
- A3243G mutation, mitochondrial DNA, heteroplasmy, reactive oxygen species, superoxide dismutase, oxidative phosphorylation, mitochondrial respiratory chain, complex I, cytochrome c oxidase, mitochondrial disease, diabetes mellitus
- MeSH terms
- Animals, Chimera, DNA, Mitochondrial, Female, Mice, Inbred C57BL, Mice, Transgenic, Superoxide Dismutase, Point Mutation, Reactive Oxygen Species, Mitochondrial Diseases, Mice
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