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Repairing Mitochondrial Dysfunction in Disease
Open accessAbstract
Mitochondria are essential organelles for many aspects of cellular homeostasis, including energy harvesting through oxidative phosphorylation. Alterations of mitochondrial function not only impact on cellular metabolism but also critically influence whole-body metabolism, health, and life span. Diseases defined by mitochondrial dysfunction have expanded from rare monogenic disorders in a strict sense to now also include many common polygenic diseases, including metabolic, cardiovascular, neurodegenerative, and neuromuscular diseases. This has led to an intensive search for new therapeutic and preventive strategies aimed at invigorating mitochondrial function by exploiting key components of mitochondrial biogenesis, redox metabolism, dynamics, mitophagy, and the mitochondrial unfolded protein response. As such, new findings linking mitochondrial function to the progression or outcome of this ever-increasing list of diseases has stimulated the discovery and development of the first true mitochondrial drugs, which are now entering the clinic and are discussed in this review.
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Metadata
- Authors
- Sorrentino, V., Menzies, K. J., & Auwerx, J.
- Affiliations
- École Polytechnique Fédérale de Lausanne (CH)
- University of Ottawa (CA)
- Venue
- Annual Review of Pharmacology and Toxicology
- Publisher
- Annual Reviews
- Published
- 2018-01-06
- Volume
- 58
- Issue
- 1
- Pages
- 353-389
- ISSN
- 0362-1642 (ISSN-L)
- 1545-4304
- OpenAlex
- W2612876306
- Cited by
- 337
- References
- 356
- Citations by year
- 2026: 25 · 2025: 59 · 2024: 55 · 2023: 36 · 2022: 46 · 2021: 43 · 2020: 36 · 2019: 27 · 2018: 10
- FWCI
- 1.87
- Type
- journal article
- Language
- en
- Open access
- green
- pure.amsterdamumc.nl
- Licence
- other-oa
- Locations
- The Annual Review of Pharmacology and Toxicology (publishedVersion)
- PubMed (publishedVersion)
- Pure Amsterdam UMC (publishedVersion, other-oa)
- National University of Singapore (submittedVersion)
- Infoscience (Ecole Polytechnique Fédérale de Lausanne) (submittedVersion, cc-by-nc-nd)
- Funders
- National Science Foundation
- University of Ottawa
- Kidney Foundation of Canada
- École Polytechnique Fédérale de Lausanne (665667)
- Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung (140780, 151019, 31003A)
- National Institutes of Health
- Canadian Institutes of Health Research
- National Institute on Aging (R01AG043930)
- 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
- Health Sciences › Medicine › Physiology (medical) › Adipose Tissue and Metabolism
- Keywords
- mitochondrial dysfunction, mitochondrial disease, mitochondrial biogenesis, redox metabolism, mitochondrial dynamics, mitophagy, mitochondrial unfolded protein response, polygenic disease, metabolic disorders, neurodegenerative diseases, neuromuscular disorders, cardiovascular disease, oxidative phosphorylation, mitochondrial therapeutics
- MeSH terms
- Animals, Organelle Biogenesis, Humans, Mitochondria, Disease Progression, Mitochondrial Diseases
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