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An ibs.GRANADA investigation identifies the serious metabolic and pathological consequences caused by coenzyme Q10 deficiency in people

ibs.GRANADA  ·  News
8th September 2022

The work, developed in part by ibs.GRANADA researchers Pilar González García and professor Luis Carlos López García, both from CIBM, shows that vanillic acid, a naturally occurring phenolic compound, is capable of correcting alterations

A study carried out by the Biomedical Research Center of the University of Granada (CIBM) ​​has tested the validity of a natural compound, vanillic acid, in animals to combat the deficiency of coenzyme Q10 (CoQ10), an essential molecule for life that It is synthesized in the cells of the organs and tissues of people.

The research, developed in part by researchers from the Intercellular Communication group of ibs.GRANADA, Pilar González García and Luis Carlos López García, shows that vanillic acid is capable of correcting the alterations in the "Q junction" and in the neuroinflammatory process derived of the lack of coenzyme Q10, increasing the survival of the animal model until it resembles the survival of control animals.

The two best known functions of CoQ10 are its participation in the process of generating useful energy for cells and its antioxidant capacity. The study has shown that coenzyme Q10 deficiency causes two important pathophysiological alterations: on the one hand, metabolic changes due to the modification in the functioning of what is called "Q junction", which are a series of proteins dependent on coenzyme Q that participate in important metabolic pathways at the cellular level; and on the other hand, the induction of reactive gliosis and neuroinflammation leading to lethal encephalopathy.

“Coenzyme Q10 deficiency occurs primarily in what we know as 'coenzyme Q10 deficiency syndrome', which occurs with a very heterogeneous clinical presentation. Likewise, it also occurs secondarily in other mitochondrial diseases or in more common pathologies such as obesity and metabolic syndrome, in certain neurodegenerative diseases or as a result of some pharmacological treatments, such as treatment with statins, among others”, explains Luis Carlos López, belonging to the Precision Medicine area of ​​the ibs.GRANADA and professor of the Department of Physiology and researcher at the CIBM.

The work carried out at the UGR also identifies potential biomarkers associated with coenzyme Q10 deficiency, which could be used in the clinic to follow the progress of the disease and evaluate the efficacy of treatments.

“Among the biomarkers that have been identified, we highlight those that are within the group of acylcarnitines, since we found a very good correlation between untreated and treated disease, and between their plasma and tissue levels. Likewise, we have also identified some metabolites of the hexosamine biosynthetic pathway as potential biomarkers, as well as several plasma proteins”, explains Luis Carlos López.

Regarding the supplement to pharmacological doses of vanillic acid or b-resorcylic acid, two phenolic compounds of natural origin, as effective treatments for this mitochondrial syndrome, their potential "must in any case be evaluated in clinical practice", the researchers conclude.

The experimental work is part of the doctoral thesis in the Biomedicine Doctoral Program of Pilar González García, contracted FPU and member of the CTS-101 group of the University of Granada. The study also has important collaborations in omics analysis, such as those of the groups of Albert Heck and Celia Berkers, from the University of Utrecht (The Netherlands), and Mohammed Bakkali, from the Department of Genetics of the University of Granada.

Bibliographic reference:

The Q-Junction and the Inflammatory Response are Critical Pathological and Therapeutic Factors in CoQ Deficiency. Redox Biology. doi: https://doi.org/10.1016/j.redox.2022.102403

Online access: https://www.sciencedirect.com/science / article / pii /S2213231722001756

Published in ibs.GRANADA
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