Open Access

Mitochondrial bioenergetics and disease in Caenorhabditis elegans

Beverley M. Dancy1,2,*,Margaret M. Sedensky1,2,Philip G. Morgan1,2
Center for Developmental Therapeutics, Seattle Children’s Research Institute, 1900 Ninth Ave, Seattle, WA, USA,
Department of Anesthesiology and Pain Medicine, University of Washington, 1959 NE Pacific St, Seattle, WA, USA
DOI: 10.2741/4305 Volume 20 Issue 2, pp.198-228
Published: 01 January 2015
(This article belongs to the Special Issue Mitochondrial bioenergetics in human health)
*Corresponding Author(s):  
Beverley M. Dancy

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.

Key words

Caenorhabditis elegans,Electron Transport,Genetics,Leigh Syndrome,Lifespan,Metabolic Disease,Mitochondrion,Reactive Oxygen Species,Respiratory Chain,Review

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Beverley M. Dancy, Margaret M. Sedensky, Philip G. Morgan. Mitochondrial bioenergetics and disease in Caenorhabditis elegans. Frontiers in Bioscience-Landmark. 2015. 20(2); 198-228.