Engineering the alternative oxidase gene to better understand and counteract mitochondrial defects: state of the art and perspectives
Open accessAbstract
Mitochondrial disorders are nowadays recognized as impinging on most areas of medicine. They include specific and widespread organ involvement, including both tissue degeneration and tumour formation. Despite the spectacular progresses made in the identification of their underlying molecular basis, effective therapy remains a distant goal. Our still rudimentary understanding of the pathophysiological mechanisms by which these diseases arise constitutes an obstacle to developing any rational treatments. In this context, the idea of using a heterologous gene, encoding a supplemental oxidase otherwise absent from mammals, potentially bypassing the defective portion of the respiratory chain, was proposed more than 10 years ago. The recent progress made in the expression of the alternative oxidase in a wide range of biological systems and disease conditions reveals great potential benefit, considering the broad impact of mitochondrial diseases. This review addresses the state of the art and the perspectives that can be now envisaged by using this strategy. Linked Articles: This article is part of a themed issue on Mitochondrial Pharmacology: Energy, Injury & Beyond. To view the other articles in this issue visit http://dx.doi.org/10.1111/bph.2014.171.issue‐8
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From Coupling Krebs cycle metabolites to signalling in immunity and cancer (2018) · cites this paper as [37]
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From Coupling Krebs cycle metabolites to signalling in immunity and cancer (2018) · cites this paper as [37]
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Coupling Krebs cycle metabolites to signalling in immunity and cancer
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What physiological role(s) does the alternative oxidase perform in animals?
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Self-citation
- Authors
- El‐Khoury, R., Kemppainen, K. K., Dufour, E., Szibor, M., Jacobs, H. T., & Rustin, P.
- Affiliations
- Inserm (FR)
- Tampere University Hospital (FI)
- Institute of Biomedical Technology Tampere University Hospital University of Tampere Tampere Finland
- University of Helsinki (FI)
- Corresponding author
- Rustin, Pierre
- Venue
- British Journal of Pharmacology
- Publisher
- Wiley
- Published
- 2014-03-28
- Volume
- 171
- Issue
- 8
- Pages
- 2243-2249
- ISSN
- 0007-1188 (ISSN-L)
- 1476-5381
- PMC
- PMC3976633
- OpenAlex
- W1917909786
- Cited by
- 56
- References
- 46
- Citations by year
- 2025: 2 · 2024: 3 · 2023: 3 · 2022: 5 · 2021: 4 · 2020: 4 · 2019: 4 · 2018: 13 · 2017: 2 · 2016: 6 · 2015: 7 · 2014: 3
- FWCI
- 0.56
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- British Journal of Pharmacology (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- 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 › Coenzyme Q10 studies and effects
- Keywords
- alternative oxidase, mitochondrial disease, electron transport chain, heterologous expression, mitochondrial disease therapy, genetic engineering, tumor formation, tissue degeneration
- MeSH terms
- Alternative Oxidase, Animals, Genetic Engineering, Humans, Mitochondria, Oxidoreductases, Plant Proteins, Mitochondrial Proteins, Mitochondrial Diseases
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