Mitochondrial bioenergetics and disease in Caenorhabditis elegans
| Autor: | Philip G. Morgan, Beverley M. Dancy, Margaret M. Sedensky |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
chemistry.chemical_classification
Reactive oxygen species Bioenergetics biology Mitochondrial disease Respiratory chain Mitochondrion medicine.disease biology.organism_classification Models Biological Mitochondria Cell biology Electron Transport Life Expectancy Mitochondrial respiratory chain chemistry Coenzyme Q – cytochrome c reductase medicine Animals Caenorhabditis elegans Energy Metabolism |
| Zdroj: | Frontiers in Bioscience. 20:198-228 |
| ISSN: | 1093-4715 1093-9946 |
| DOI: | 10.2741/4305 |
| Popis: | Simple multicellular animal model systems are central to studying the complex mechanisms underlying a bewildering array of diseases involving dysfunctional mitochondria. Mutant nuclear- and mitochondrial-encoded subunits of the Caenorhabditis elegans mitochondrial respiratory chain (MRC) have been investigated, including GAS-1, NUO-1, NUO-6, MEV-1, SDHB-1, CLK-1, ISP-1, CTB-1, and ATP-2. These, as well as proteins that modify the MRC indirectly, have been studied on the molecular, cellular, and organismal levels through the variety of experimental approaches that are readily achievable in C. elegans. In C. elegans, MRC dysfunction can mimic signs and symptoms observed in human patients with primary mitochondrial disorders, such as neuromuscular deficits, developmental delay, altered anesthetic sensitivity, and increased lactate levels. Antioxidant dietary supplements, coenzyme Q substitutes, and flavin cofactors have been explored as potential therapeutic strategies. Furthermore, mutants with altered longevity have proved useful for probing the contributions of bioenergetics, reactive oxygen species, and stress responses to the process of aging. C. elegans will undoubtedly continue to provide a useful system in which to explore unanswered questions in mitochondrial biology and disease. |
| Databáze: | OpenAIRE |
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