Secondary Coenzyme Q 10 Deficiencies in Oxidative Phosphorylation (OXPHOS) and Non-OXPHOS Disorders
Abstract
We evaluated the coenzyme Q₁₀ (CoQ) levels in patients who were diagnosed with mitochondrial oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders (n=72). Data from the 72 cases in this study revealed that 44.4% of patients showed low CoQ concentrations in either their skeletal muscle or skin fibroblasts. Our findings suggest that secondary CoQ deficiency is a common finding in OXPHOS and non-OXPHOS disorders. We hypothesize that cases of CoQ deficiency associated with OXPHOS defects could be an adaptive mechanism to maintain a balanced OXPHOS, although the mechanisms explaining these deficiencies and the pathophysiological role of secondary CoQ deficiency deserves further investigation.
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A lack of CoQ10 has been related to several conditions, including aging and neurological disorders.
“A lack of CoQ10 has been related to several conditions, including aging and neurological disorders [83,84,85,86,87,88,89] (Table 1).”
From The Role of Antioxidants in the Interplay between Oxidative Stress and Senescence (2022) · cites this paper as [84]
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References 42 · Cited by 72
- Authors
- Yubero, D., Montero, R., Martín, M. A., Montoya, J., Ribes, A., Grazina, M., Trevisson, E., Rodriguez-Aguilera, J. C., Hargreaves, I. P., Salviati, L., Navas, P., Artuch, R., Jou, C., Jimenez-Mallebrera, C., Nascimento, A., Pérez-Dueñas, B., Ortez, C., Ramos, F., Colomer, J., O’Callaghan, M., Pineda, M., García-Cazorla, A., Espinós, C., Ruiz, A., Macaya, A., Marcé-Grau, A., Garcia-Villoria, J., Arias, A., Emperador, S., Ruiz-Pesini, E., Lopez-Gallardo, E., Neergheen, V., Simões, M., Diogo, L., Blázquez, A., González-Quintana, A., Delmiro, A., Domínguez-González, C., Arenas, J., García-Silva, M. T., Martín, E., Quijada, P., Hernández-Laín, A., Morán, M., Rivas Infante, E., Ávila Polo, R., Paradas Lópe, C., Bautista Lorite, J., Martínez Fernández, E. M., Cortés, A. B., Sánchez-Cuesta, A., Cascajo, M. V., Alcázar, M., & Brea-Calvo, G.
- Affiliations
- Instituto de Salud Carlos III (ES)
- Universidad de Zaragoza (ES)
- Centro de Neurociências e Biologia Celular (PT)
- Città della Speranza Foundation (IT)
- National Hospital for Neurology and Neurosurgery (GB)
- Consejo Superior de Investigaciones Científicas (ES)
- Hospital Universitario Son Espases (ES)
- Vall d'Hebron Institut de Recerca (ES)
- Centro de Investigacion Principe Felipe (ES)
- Corresponding author
- Artuch, Rafael
- ORCID
- Montero, Raquel
- Martín, Miguel A.
- Montoya, Julio
- Grazina, Manuela
- Trevisson, Eva
- Hargreaves, Iain P.
- Salviati, Leonardo
- Navas, Plácido
- Artuch, Rafael
- Jou, Cristina
- Jimenez-Mallebrera, Cecilia
- Ortez, Carlos
- Ramos, Federico
- Colomer, Jaume
- O’Callaghan, Mar
- Pineda, Mercè
- Macaya, Alfons
- Emperador, Sonia
- Simões, Marta
- Blázquez, Alberto
- González-Quintana, Adrián
- Delmiro, Aitor
- Morán, María
- Cortés, Ana B.
- Cascajo, Maria V.
- Venue
- Mitochondrion
- Publisher
- Elsevier BV
- Published
- 2016-09
- Volume
- 30
- Pages
- 51-58
- ISSN
- 1567-7249 (ISSN-L)
- OpenAlex
- W2465830552
- Cited by
- 72
- References
- 42
- Citations by year
- 2026: 1 · 2025: 3 · 2024: 4 · 2023: 4 · 2022: 7 · 2021: 17 · 2020: 13 · 2019: 8 · 2018: 9 · 2017: 6
- FWCI
- 3.99
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Mitochondrion (publishedVersion)
- PubMed (publishedVersion)
- DIGITAL.CSIC (Spanish National Research Council (CSIC)) (submittedVersion)
- Padua Research Archive (University of Padova) (submittedVersion)
- Zaguan (Universidad de Zaragoza) (submittedVersion)
- Funders
- Instituto de Salud Carlos III (PI12/01683, PI14/00005, PI14/00028, PI14/01962)
- Explora Ciencia Program (SAF2013-50139-EXP)
- Universidad del Gobierno de Aragón
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Coenzyme Q10 studies and effects
- 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
- Keywords
- coenzyme Q10 deficiency, oxidative phosphorylation disorders, mitochondrial disease, skeletal muscle, dermal fibroblasts, adaptive mechanisms
- MeSH terms
- Adolescent, Adult, Child, Child, Preschool, Female, Humans, Infant, Infant, Newborn, Male, Middle Aged, Muscles, Oxidative Phosphorylation, Skin, Ubiquinone, Cohort Studies, Prevalence, Mitochondrial Diseases, Young Adult
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