ArticleMolecular therapy : the journal of the American Society of Gene Therapy2013
Reducing mitochondrial ROS improves disease-related pathology in a mouse model of ataxia-telangiectasia.
Article in Molecular therapy : the journal of the American Society of Gene Therapy, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
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Who cites it
41 citing papers in PubMed, 68 citations in OpenAlex.
- Recent Advances in Therapy for the Neurodegenerative Disorder Ataxia-Telangiectasia.International journal of molecular sciences · 2026Review
- Mitochondria-Targeted Human Catalase in the Mouse Longevity MCAT Model Mitigates Head-Tilt Bedrest-Induced Neuro-Inflammation in the Hippocampus.Life (Basel, Switzerland) · 2022Article
- Electrophilic Aldehyde 4-Hydroxy-2-Nonenal Mediated Signaling and Mitochondrial Dysfunction.Biomolecules · 2022Review
- Global metabolic alterations in colorectal cancer cells during irinotecan-induced DNA replication stress.Cancer & metabolism · 2022Article
- Defining roles of specific reactive oxygen species (ROS) in cell biology and physiology.Nature reviews. Molecular cell biology · 2022Review
- Metabolic Stress and Mitochondrial Dysfunction in Ataxia-Telangiectasia.Antioxidants (Basel, Switzerland) · 2022Review
- Cellular functions of the protein kinase ATM and their relevance to human disease.Nature reviews. Molecular cell biology · 2021Review
- An anaplerotic approach to correct the mitochondrial dysfunction in ataxia-telangiectasia (A-T).Molecular metabolism · 2021Article
- Article
- Ataxia Telangiectasia Mutated Protein Kinase: A Potential Master Puppeteer of Oxidative Stress-Induced Metabolic Recycling.Oxidative medicine and cellular longevity · 2021Review
- Peroxisomes in Immune Response and Inflammation.International journal of molecular sciences · 2019Review
- Redox Mechanisms in Neurodegeneration: From Disease Outcomes to Therapeutic Opportunities.Antioxidants & redox signaling · 2019Review
- Histone H2AX promotes neuronal health by controlling mitochondrial homeostasis.Proceedings of the National Academy of Sciences of the United States of America · 2019Article
- The Emerging Role of Estrogens in Thyroid Redox Homeostasis and Carcinogenesis.Oxidative medicine and cellular longevity · 2019Review
- Mitochondrial redox sensing by the kinase ATM maintains cellular antioxidant capacity.Science signaling · 2018Article
- Inflammation, a significant player of Ataxia-Telangiectasia pathogenesis?Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2018Review
- Spontaneous DNA damage to the nuclear genome promotes senescence, redox imbalance and aging.Redox biology · 2018Article
- Regulation of redox balance in cancer and T cells.The Journal of biological chemistry · 2018Review
- Histone H2AX deficiency causes neurobehavioral deficits and impaired redox homeostasis.Nature communications · 2018Article
- ATM directs DNA damage responses and proteostasis via genetically separable pathways.Science signaling · 2018Article
Corrections and comments
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Authors and funding
5 authors at 1 institution in 1 country.
Funding
Abstract
The disease ataxia-telangiectasia (A-T) has no cure and few treatment options. It is caused by mutations in the ATM kinase, which functions in the DNA-damage response and redox sensing. In addition to severe cerebellar degeneration, A-T pathology includes cancer predisposition, sterility, immune system dysfunction, and bone marrow abnormalities. These latter phenotypes are recapitulated in the ATM null (ATM(-/-)) mouse model of the disease. Since oxidative stress and mitochondrial dysfunction are implicated in A-T, we determined whether reducing mitochondrial reactive oxygen species (ROS) via overexpression of catalase targeted to mitochondria (mCAT) alleviates A-T-related pathology in ATM(-/-) mice. We found that mCAT has many beneficial effects in this context, including reduced propensity to develop thymic lymphoma, improved bone marrow hematopoiesis and macrophage differentiation in vitro, and partial rescue of memory T-cell developmental defects. Our results suggest that positive effects observed on cancer development may be linked to mCAT reducing mitochondrial ROS, lactate production, and TORC1 signaling in transforming double-positive cells, whereas beneficial effects in memory T cells appear to be TORC1-independent. Altogether, this study provides proof-of-principle that reducing mitochondrial ROS production per se may be therapeutic for the disease, which may have advantages compared with more general antioxidant strategies.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.