IMR Press / FBL / Volume 20 / Issue 2 / DOI: 10.2741/4305

Frontiers in Bioscience-Landmark (FBL) is published by IMR Press from Volume 26 Issue 5 (2021). Previous articles were published by another publisher on a subscription basis, and they are hosted by IMR Press on as a courtesy and upon agreement with Frontiers in Bioscience.


Mitochondrial bioenergetics and disease in Caenorhabditis elegans

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1 Center for Developmental Therapeutics, Seattle Children’s Research Institute, 1900 Ninth Ave, Seattle, WA, USA,
2 Department of Anesthesiology and Pain Medicine, University of Washington, 1959 NE Pacific St, Seattle, WA, USA
Front. Biosci. (Landmark Ed) 2015, 20(2), 198–228;
Published: 1 January 2015

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.

Caenorhabditis elegans
Electron Transport
Leigh Syndrome
Metabolic Disease
Reactive Oxygen Species
Respiratory Chain
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