Cited by 390Mitochondrial Function and PathologyAutophagy in Disease and TherapyGenetics, Aging, and Longevity in Model Organisms

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Cell Biology of the Mitochondrion

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van der Bliek, Alexander M, Sedensky, Margaret M, Morgan, Phil G·2017·Genetics

DOI 10.1534/genetics.117.300262PMC PMC5676242

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Abstract

Mitochondria are best known for harboring pathways involved in ATP synthesis through the tricarboxylic acid cycle and oxidative phosphorylation. Major advances in understanding these roles were made with Caenorhabditiselegans mutants affecting key components of the metabolic pathways. These mutants have not only helped elucidate some of the intricacies of metabolism pathways, but they have also served as jumping off points for pharmacology, toxicology, and aging studies. The field of mitochondria research has also undergone a renaissance, with the increased appreciation of the role of mitochondria in cell processes other than energy production. Here, we focus on discoveries that were made using C. elegans, with a few excursions into areas that were studied more thoroughly in other organisms, like mitochondrial protein import in yeast. Advances in mitochondrial biogenesis and membrane dynamics were made through the discoveries of novel functions in mitochondrial fission and fusion proteins. Some of these functions were only apparent through the use of diverse model systems, such as C. elegans. Studies of stress responses, exemplified by mitophagy and the mitochondrial unfolded protein response, have also benefitted greatly from the use of model organisms. Recent developments include the discoveries in C. elegans of cell autonomous and nonautonomous pathways controlling the mitochondrial unfolded protein response, as well as mechanisms for degradation of paternal mitochondria after fertilization. The evolutionary conservation of many, if not all, of these pathways ensures that results obtained with C. elegans are equally applicable to studies of human mitochondria in health and disease.

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Mitochondrial genetics
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The effect of different ubiquinones on lifespan in Caenorhabditis elegans
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Mitochondrial proteins: from biogenesis to functional networks
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“Normal cells (except erythrocytes and skeletal muscle cells during high intensity exercise) depend on OXPHOS for ATP production from glucose; on the contrary, cancer cells obtain their ATP by glycolysis and the final conversion of glucose to lactate (40).”
Background
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Authors: van der Bliek, A. M., Sedensky, M. M., & Morgan, P. G.
Affiliations: Department of Biological Chemistry, Jonsson Comprehensive Cancer Center and Molecular Biology Institute, David Geffen School of Medicine at UCLA, Los Angeles, California 90024
University of Washington (US)
Department of Anesthesiology and Pain Medicine, University of Washington and Center for Developmental Therapeutics, Seattle Children’s Research Institute, Washington 98101
ORCID: Sedensky, Margaret M
Venue: Genetics
Publisher: Oxford University Press (OUP)
Published: 2017-10-25
Volume: 207
Issue: 3
Pages: 843-871
ISSN: 0016-6731 (ISSN-L)
1943-2631
DOI: 10.1534/genetics.117.300262
PMC: PMC5676242
OpenAlex: W2765579241
Cited by: 388
References: 234
Citations by year: 2026: 22 · 2025: 45 · 2024: 44 · 2023: 58 · 2022: 60 · 2021: 59 · 2020: 62 · 2019: 26 · 2018: 11
FWCI: 2.53
Type: journal article
Language: en
Open access: bronze
doi.org
Licence: academic.oup.com/journals/pages/open_access/funder_policies/chorus/standard_publication_model (vor)
Locations: Genetics (publishedVersion)
Europe PMC (PubMed Central) (publishedVersion)
PubMed Central (publishedVersion)
PubMed (publishedVersion)
eScholarship (California Digital Library) (submittedVersion)
Funders: National Institutes of Health
National Institute of General Medical Sciences (U01GM109764, R01GM118514, R01GM105696)
Topics: Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Autophagy in Disease and Therapy
Life Sciences › Biochemistry, Genetics and Molecular Biology › Aging › Genetics, Aging, and Longevity in Model Organisms
Keywords: mitochondria, TCA cycle, oxidative phosphorylation, mitochondrial protein import, mitochondrial biogenesis, mitochondrial fission, mitochondrial fusion, mitophagy, mitochondrial unfolded protein response, stress response, aging
MeSH terms: Animals, Organelle Biogenesis, Citric Acid Cycle, Electron Transport, Mitochondria

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