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Updated: Sep 2, 2025
Author Spotlight: Decoding Mitochondrial Aging
Published on: June 30, 2023
mtDNA Maintenance and Alterations in the Pathogenesis of Neurodegenerative Diseases
Dehao Shang1, Minghao Huang1, Biyao Wang2
1Center of Implant Dentistry, School and Hospital of Stomatology, China Medical University, Liaoning Provincial Key Laboratory of Oral Diseases, Shenyang, China.
Abstract:
Considerable evidence indicates that the semiautonomous organelles mitochondria play key roles in the progression of many neurodegenerative disorders. Mitochondrial DNA (mtDNA) encodes components of the OXPHOS complex but mutated mtDNA accumulates in cells with aging, which mirrors the increased prevalence of neurodegenerative diseases. This accumulation stems not only from the misreplication of mtDNA and the highly oxidative environment but also from defective mitophagy after fission. In this review, we focus on several pivotal mitochondrial proteins related to mtDNA maintenance (such as ATAD3A and TFAM), mtDNA alterations including mtDNA mutations, mtDNA elimination, and mtDNA release-activated inflammation to understand the crucial role played by mtDNA in the pathogenesis of neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, amyotrophic lateral sclerosis, and Huntington's disease. Our work outlines novel therapeutic strategies for targeting mtDNA.
Insights
Mitochondrial DNA (mtDNA) mutations accumulate with aging, driving neurodegenerative diseases like Alzheimer's and Parkinson's. Targeting mtDNA offers novel therapeutic strategies for these conditions.
Area of Science:
- Neuroscience
- Genetics
- Cell Biology
Background:
- Mitochondria are crucial organelles implicated in neurodegenerative disease progression.
- Mitochondrial DNA (mtDNA) mutations increase with age, correlating with neurodegenerative disease prevalence.
- Defective mtDNA maintenance, replication, and mitophagy contribute to mtDNA accumulation.
Conclusions:
- mtDNA alterations are central to the pathogenesis of major neurodegenerative disorders.
- Understanding mtDNA dynamics provides insights into disease mechanisms.
- Targeting mtDNA presents a promising therapeutic avenue for neurodegenerative diseases.
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