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Mitochondrial Respiratory Complex I: Structure, Function and Implication in Human Diseases
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Mitochondria are ubiquitous organelles in eukaryotic cells whose primary function is to generate energy supplies in the form of ATP through oxidative phosphorylation. As the entry point for most electrons into the respiratory chain, NADH:ubiquinone oxidoreductase, or complex I, is the largest and least understood component of the mitochondrial oxidative phosphorylation system. Substantial progress has been made in recent years in understanding its subunit composition, its assembly, the interaction among complex I and other respiratory components, and its role in oxidative stress and apoptosis. This review provides an updated overview of the structure of complex I, as well as its cellular functions, and discusses the implication of complex I dysfunction in various human diseases.
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“An undesired effect of the redox reactions occurring in mitochondria is the generation of reactive oxygen species [27].”
From Superoxide Anion Chemistry—Its Role at the Core of the Innate Immunity (2023) · cites this paper as [27]
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Complex I from the fungus Neurospora crassa
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The mutant human ND4 subunit of complex I induces optic neuropathy in the mouse
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Mitochondrial DNA damage in non-melanoma skin cancer
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From NADH to ubiquinone in Neurospora mitochondria
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Substrate-induced Conformational Change in Bacterial Complex I
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LHON and Other Optic Nerve Atrophies: The Mitochondrial Connection
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GRIM-19 Is Essential for Maintenance of Mitochondrial Membrane Potential
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Suppression of complex I gene expression induces optic neuropathy
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Mutated ND2 impairs mitochondrial complex I assembly and leads to Leigh Syndrome
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Generation of Reactive Oxygen Species by Mitochondrial Complex I: Implications in Neurodegeneration
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Mitochondrial complex I activity is impaired during HIV-1-induced T-cell apoptosis
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The mechanism of proton and electron transport in mitochondrial complex I
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Oxidative Phosphorylation Enzymes in Normal and Neoplastic Cell Growth
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Mitochondrial complex I function modulates volatile anesthetic sensitivity in C. elegans
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Development and Characterization of a Conditional Mitochondrial Complex I Assembly System
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Biogenesis of Respiratory Complex I
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Species-specific and Mutant MWFE Proteins
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NADH dehydrogenases: from basic science to biomedicine
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In vivo dissection of the mitochondrial respiratory NADH:ubiquinone oxidoreductase (complex I)
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Self-citationGenetic and Functional Analysis of Mitochondrial DNA‐Encoded Complex I Genes
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Self-citationMitochondrial Complex I Function Affects Halothane Sensitivity in Caenorhabditis elegans
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On Complex I and Other NADH:Ubiquinone Reductases of Neurospora crassa Mitochondria
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Nuclear Suppression of Mitochondrial Defects in Cells without the ND6 Subunit
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Self-citationEscherichia coli NADH dehydrogenase I, a minimal form of the mitochondrial complex I
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Self-citationRestoration of Mitochondrial Function in Cells with Complex I Deficiency
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Genetic and Functional Analysis of Mitochondrial DNA-Encoded Complex I Genes
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4 of the 161 references couldn’t be matched to a known paper.
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The sites and topology of mitochondrial superoxide production
Brand, M. D. (2010) · Experimental Gerontology · doi:10.1016/j.exger.2010.01.003 · Cited by 1,217
Reactive Oxygen Species: A Key Hallmark of Cardiovascular Disease
Panth, N., Paudel, K. R., & Parajuli, K. (2016) · Advances in Medicine · doi:10.1155/2016/9152732 · Cited by 415
Superoxide Anion Chemistry—Its Role at the Core of the Innate Immunity
Andrés, C. M. C., Pérez de la Lastra, J. M., Andrés Juan, C., Plou, F. J., & Pérez-Lebeña, E. (2023) · International Journal of Molecular Sciences · doi:10.3390/ijms24031841 · Cited by 342
The Roles of ROS in Cancer Heterogeneity and Therapy
Júnior, P. L. D. S., Câmara, D. A. D., Porcacchia, A. S., Fonseca, P. M. M., Jorge, S. D., Araldi, R. P., & Ferreira, A. K. (2017) · Oxidative Medicine and Cellular Longevity · doi:10.1155/2017/2467940 · Cited by 298
Jiang, L., Yin, X., Chen, Y., Chen, Y., Jiang, W., Zheng, H., Huang, F., Liu, B., Zhou, W., Qi, L., et al. (2020) · Theranostics · doi:10.7150/thno.43895 · Cited by 217
Metadata
- Authors
- Sharma, L., Lu, J., & Bai, Y.
- Affiliations
- The University of Texas at San Antonio Health Science Center (US)
- Venue
- Current Medicinal Chemistry
- Publisher
- Bentham Science Publishers Ltd.
- Published
- 2009-04-01
- Volume
- 16
- Issue
- 10
- Pages
- 1266-1277
- ISSN
- 0929-8673 (ISSN-L)
- PMC
- PMC4706149
- OpenAlex
- W2126962886
- Cited by
- 438
- References
- 161
- Citations by year
- 2026: 41 · 2025: 51 · 2024: 69 · 2023: 52 · 2022: 48 · 2021: 50 · 2020: 42 · 2019: 22 · 2018: 16 · 2017: 15 · 2016: 2 · 2015: 2 · 2014: 1 · 2013: 7 · 2012: 8
- FWCI
- 0.51
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Current Medicinal Chemistry (publishedVersion)
- PubMed (publishedVersion)
- Europe PMC (PubMed Central) (acceptedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institutes of Health
- National Institute on Aging (R01AG025223)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Structural Biology › Enzyme Structure and Function
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- Keywords
- mitochondrial complex I, complex I, mitochondrial respiratory chain, oxidative phosphorylation, complex I assembly, subunit composition, oxidative stress, apoptosis, complex I dysfunction, human disease
- MeSH terms
- Animals, Disease, Humans, Apoptosis, Reactive Oxygen Species, Electron Transport Complex I
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
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