Human complex I deficiency: Clinical spectrum and involvement of oxygen free radicals in the pathogenicity of the defect
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The second enzyme is located near the mitochondrial inner membrane. This is a SOD that binds manganese in its catalytic centre (SOD2 or Mn-SOD).
“The second enzyme is located near the mitochondrial inner membrane. This is a SOD that binds manganese in its catalytic centre (SOD2 or Mn-SOD) [71,72].”
From Superoxide Anion Chemistry—Its Role at the Core of the Innate Immunity (2023) · cites this paper as [71]
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Smeitink, J. A. M., van den Heuvel, L., Koopman, W. J. H., Nijtmans, L. G. J., Ugalde, C., & Willems, P. H. G. M. (2004) · Current Neurovascular Research · doi:10.2174/1567202043480224 · Cited by 59 · see citing works
Mitochondrial Respiratory Chain Diseases and Mutations in Nuclear DNA: A Promising Start?
Sue, C. M., & Schon, E. A. (2000) · Brain Pathology · doi:10.1111/j.1750-3639.2000.tb00276.x · Cited by 57 · see citing works
Mitochondrial Myopathies and the Role of the Pathologist in the Molecular Era
Vogel, H. S. (2001) · Journal of Neuropathology & Experimental Neurology · doi:10.1093/jnen/60.3.217 · Cited by 59 · see citing works
Grad, L. I., & Lemire, B. D. (2006) · Biochimica et Biophysica Acta (BBA) - Bioenergetics · doi:10.1016/j.bbabio.2005.11.009 · Cited by 57 · see citing works
Secondary metabolic effects in complex I deficiency
Esteitie, N., Hinttala, R., Wibom, R., Nilsson, H., Hance, N., Naess, K., Fahnehjelm, K. T., von Döbeln, U., Majamaa, K., & Larsson, N. (2005) · Annals of Neurology · doi:10.1002/ana.20570 · Cited by 57 · see citing works
Open-minded scepticism: inferring the causal mechanisms of human ageing from genetic perturbations
de Magalhães, J. P. (2005) · Ageing Research Reviews · doi:10.1016/j.arr.2004.05.003 · Cited by 54 · see citing works
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Inhibition of the Mitochondrial Respiratory Chain by Phenylalanine in Rat Cerebral Cortex
Rech, V. C., Feksa, L. R., Dutra‐Filho, C. S., Wyse, Â. T. D. S., Wajner, M., & Wannmacher, C. M. D. (2002) · Neurochemical Research · doi:10.1023/a:1015529511664 · Cited by 51 · see citing works
- Authors
- Robinson, B. H.
- Affiliations
- University of Toronto (CA)
- Corresponding author
- Robinson, Brian H
- Venue
- Biochimica et Biophysica Acta (BBA) - Bioenergetics
- Publisher
- Elsevier BV
- Published
- 1998-05
- Volume
- 1364
- Issue
- 2
- Pages
- 271-286
- ISSN
- 0005-2728 (ISSN-L)
- OpenAlex
- W2067124266
- Cited by
- 223
- References
- 115
- Citations by year
- 2025: 3 · 2024: 2 · 2023: 2 · 2022: 5 · 2021: 5 · 2020: 1 · 2019: 7 · 2018: 5 · 2017: 4 · 2016: 6 · 2015: 6 · 2014: 5 · 2013: 6 · 2012: 6
- FWCI
- 1.13
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Licence
- Locations
- Biochimica et Biophysica Acta (BBA) - Bioenergetics (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Heart and Stroke Foundation of Canada
- Australian Government
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- Keywords
- complex I deficiency, mitochondrial disease, clinical spectrum, oxygen free radicals, oxidative stress, lactic acidosis, optic neuropathy, exercise intolerance, mitochondrial DNA, nuclear DNA
- MeSH terms
- Free Radicals, Humans, Metabolism, Inborn Errors, NAD(P)H Dehydrogenase (Quinone)
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