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Cell Biology of the Mitochondrion
Open accessAbstract
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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“Mitochondria are the principal source of ATP, which is formed by oxidative phosphorylation following electron flow through highly integrated protein complexes; however, mitochondria are also involved in many other functions as well, which, among others, include apoptosis, protein synthesis, and fatty acid oxidation [14,15].”
From Mitochondrial Oxidative and Nitrosative Stress and Alzheimer Disease (2020) · cites this paper as [14]
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Metadata
- 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
- 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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