Cited by 54Mitochondrial Function and PathologyGenetics, Aging, and Longevity in Model OrganismsAutophagy in Disease and Therapy

C. elegans as a model organism for human mitochondrial associated disorders

Maglioni, Silvia, Ventura, Natascia·2016·Mitochondrion

Abstract

Mitochondria are small cytoplasmic organelles whose most important function is to provide the energy required by our cells and organism to live. To maintain an adequate mitochondrial homeostasis cells possess numerous mitochondrial quality controls and protective compensatory pathways, which can be activated to cope with a certain degree of mitochondrial dysfunction. However, when the mitochondrial damage is too severe and these defensive mechanisms are not anymore sufficient to deal with it, pathological signs arise. In the past few decades numerous genetic disorders ascribed to severe mitochondrial defects have been recognized with variable onset and symptomatology ranging from neuromuscular degeneration to cancer syndromes. Unfortunately, to date, only symptomatic and no curative therapies exist for most of these devastating, life-threatening disorders. Model organisms, and especially the nematode Caenorhabditis elegans, with its sequenced and highly conserved genome, and a simple but well-characterized nervous system, have enormously contributed in the past years to gain insight into the pathogenesis and treatment of different diseases. Here, we will summarize some of the advantages offered by the nematode system to model neurodegenerative diseases associated with mitochondrial electron transport chain defects and screen for therapeutic interventions.

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Crossref · 10.1016/j.mito.2016.02.003

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  • The round worm Caenorhabditis elegans is used to investigate metabolic pathways and has been instrumental in elucidating key genetic regulators of metabolism in relation to lifespan.

    “The round worm Caenorhabditis elegans, for instance, is used to investigate metabolic pathways and has been instrumental in elucidating key genetic regulators of metabolism in relation to lifespan (36, 293, 294).”

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Authors
Maglioni, S., & Ventura, N.
Affiliations
Leibniz Institute of Environmental Medicine (DE)
Corresponding author
Ventura, Natascia
Venue
Mitochondrion
Publisher
Elsevier BV
Published
2016-09
Volume
30
Pages
117-125
ISSN
1567-7249 (ISSN-L)
OpenAlex
W2274068909
Cited by
54
References
119
Citations by year
2026: 2 · 2025: 3 · 2024: 7 · 2023: 4 · 2022: 7 · 2021: 4 · 2020: 5 · 2019: 5 · 2018: 6 · 2017: 9 · 2016: 2
FWCI
0.66
Type
journal article
Language
en
Open access
closed
Locations
  • Mitochondrion (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Aging › Genetics, Aging, and Longevity in Model Organisms
  • Health Sciences › Medicine › Epidemiology › Autophagy in Disease and Therapy
Keywords
Caenorhabditis elegans, mitochondrial disease, mitochondrial dysfunction, mitochondrial quality control, neurodegenerative diseases, model organisms, therapeutic screening, mitochondrial homeostasis
MeSH terms
Animals, Disease Models, Animal, Humans, Muscular Diseases, Caenorhabditis elegans, Neurodegenerative Diseases, Mitochondrial Diseases

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