akusherstvoObstetrics, Gynecology and ReproductionАкушерство, Гинекология и Репродукция2313-73472500-3194IRBIS LLC10.17749/2313-7347/ob.gyn.rep.2024.541akusherstvo-2136Research ArticleREVIEW ARTICLESНАУЧНЫЕ ОБЗОРЫCoenzyme Q10 and embryonic development: a potential role in reproductive medicineКоэнзим Q10 и эмбриональное развитие: потенциальная роль в репродуктивной медицинеhttps://orcid.org/0009-0004-2111-467XХамадьяноваА. У.KhamadyanovaA. U.

Хамадьянова Аида Ульфатовна, к.м.н.

450008, Уфа, ул. Ленина, д. 3

Aida U. Khamadyanova, MD, PhD

3 Lenin Str., Ufa 450008

aida.khamadyanova@mail.ruhttps://orcid.org/0009-0004-2111-467XМаннановР. М.MannanovR. M.

Маннанов Руслан Маратович

450008, Уфа, ул. Ленина, д. 3

Ruslan M. Mannanov

3 Lenin Str., Ufa 450008

https://orcid.org/0009-0000-2659-7683СмаковаД. М.SmakovaD. M.

Смакова Дина Марселевна

450008, Уфа, ул. Ленина, д. 3

Dina M. Smakova

3 Lenin Str., Ufa 450008

https://orcid.org/0009-0008-7398-9098МусаеваФ. И.MusaevaF. I.

Мусаева Фатима Ибрагимовна

414000 Астрахань, Бакинская ул., д 121

Fatima I. Musaeva

121 Bakinskaya Str., Astrakhan 414000

https://orcid.org/0009-0001-7221-1603БеделовД. Г.BedelovD. G.

Беделов Давид Гамидович

414000 Астрахань, Бакинская ул., д 121

David G. Bedelov

121 Bakinskaya Str., Astrakhan 414000

https://orcid.org/0009-0006-9047-4366ИбрагимовА. Э.IbragimovA. E.

Ибрагимов Аллахверди Эльбрусович

414000 Астрахань, Бакинская ул., д 121

Allakhverdi E. Ibragimov

121 Bakinskaya Str., Astrakhan 414000

https://orcid.org/0009-0004-5789-6706РусиноваА. А.RusinovaA. A.

Анна Анатольевна Русинова

194100 Санкт-Петербург, Литовская ул., д. 2

Anna A. Rusinova

2 Litovskaya Str., Saint Petersburg 194100

https://orcid.org/0009-0007-0743-5065СалиховаМ. М.SalikhovaM. M.

Салихова Маргарита Маратовна

450008, Уфа, ул. Ленина, д. 3

Margarita M. Salikhova

3 Lenin Str., Ufa 450008

https://orcid.org/0009-0002-1109-8907ШтукатуроваС. В.ShtukaturovaS. V.

Штукатурова Светлана Владимировна

394036 Воронеж, Студенческая ул., д. 10

Svetlana V. Shtukaturova

10 Studencheskaya Str., Voronezh 394036

https://orcid.org/0009-0004-6281-5050ДорошенкоТ. В.DoroshenkoT. V.

Дорошенко Татьяна Витальевна

350063 Краснодар, ул. Митрофана Седина, д. 4

Tatyana V. Doroshenko

4 Mitrofana Sedina Str., Krasnodar 350063

https://orcid.org/0009-0009-3052-9347ФаттаховаМ. В.FattakhovaM. V.

Фаттахова Майя Вилевна

450008, Уфа, ул. Ленина, д. 3

Maya V. Fattakhova

3 Lenin Str., Ufa 450008

https://orcid.org/0009-0009-2477-6034РахимоваМ. К.RakhimovaM. K.

Рахимова Мижгона Камолджоновна

450008, Уфа, ул. Ленина, д. 3

Mizhgona K. Rakhimova

3 Lenin Str., Ufa 450008

https://orcid.org/0009-0004-4046-5846МариноваЛ. Р.MarinovaL. R.

Маринова Лилия Руслановна

117997 Москва, ул. Островитянова, 1

Liliya R. Marinova

1 Ostrovityanova Str., Moscow 117997

ФГБОУ ВО «Башкирский государственный медицинский университет» Министерства здравоохранения Российской ФедерацииРоссияBashkir State Medical University, Health Ministry of Russian FederationRussian FederationФГБОУ ВО «Астраханский государственный медицинский университет» Министерства здравоохранения Российской ФедерацииРоссияAstrakhan State Medical University, Health Ministry of Russian FederationRussian FederationФГБОУ ВО «Санкт-Петербургский государственный педиатрический медицинский университет» Министерства здравоохранения Российской ФедерацииРоссияSaint Petersburg State Pediatric Medical University, Health Ministry of Russian FederationRussian FederationФГБОУ ВО «Воронежский государственный медицинский университет имени Н.Н. Бурденко» Министерства здравоохранения Российской ФедерацииРоссияBurdenko Voronezh State Medical University, Health Ministry of Russian FederationRussian FederationФГБОУ ВО «Кубанский государственный медицинский университет» Министерства здравоохранения Российской ФедерацииРоссияKuban State Medical University, Health Ministry of Russian FederationRussian FederationФГАОУ ВО «Российский национальный исследовательский медицинский университет имени Н.И. Пирогова» Министерства здравоохранения Российской ФедерацииРоссияPirogov Russian National Research Medical University, Health Ministry of Russian FederationRussian Federation202413082024185720734Copyright © Khamadyanova A.U., Mannanov R.M., Smakova D.M., Musaeva F.I., Bedelov D.G., Ibragimov A.E., Rusinova A.A., Salikhova M.M., Shtukaturova S.V., Doroshenko T.V., Fattakhova M.V., Rakhimova M.K., Marinova L.R., 20242024Хамадьянова А.У., Маннанов Р.М., Смакова Д.М., Мусаева Ф.И., Беделов Д.Г., Ибрагимов А.Э., Русинова А.А., Салихова М.М., Штукатурова С.В., Дорошенко Т.В., Фаттахова М.В., Рахимова М.К., Маринова Л.Р.Khamadyanova A.U., Mannanov R.M., Smakova D.M., Musaeva F.I., Bedelov D.G., Ibragimov A.E., Rusinova A.A., Salikhova M.M., Shtukaturova S.V., Doroshenko T.V., Fattakhova M.V., Rakhimova M.K., Marinova L.R.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.gynecology.su/jour/article/view/2136

Maternal mitochondria provide energy to the embryo through oxidative phosphorylation before blastocyst implantation, where intracellular energy is mainly supplied by glycolysis. Thus, it is obvious that mitochondria play a crucial role in providing energy for embryogenesis. Coenzyme Q10 (CoQ10) is a powerful endogenous membrane-localized antioxidant that protects circulating lipoproteins from lipid peroxidation. The results of several recent clinical studies have shown that exogenous CoQ10 supplements exert antioxidant effects and may be a potential therapy to reduce oxidative stress. CoQ10 deficiency increases the risk of impaired embryonic development; however, this relationship remains unclear. Given that CoQ10 level is influenced by enzymes involved in its synthesis, it is difficult to say whether the disorders are caused by CoQ10 deficiency or directly result from defects in the target gene. It has been shown that in the absence of CoQ10, ATP synthesis decreases in parallel with increased oxidative stress in mitochondria, two biological events which affect embryonic development. The review highlights the importance of CoQ10 as an antioxidant for improving egg quality, and also emphasizes its key role in embryonic development. It is necessary to conduct further studies aimed at studying metabolic changes during embryogenesis, as well as the mechanism of CoQ10 effects.

Материнские митохондрии обеспечивают энергией эмбрион посредством окислительного фосфорилирования перед имплантацией бластоцисты. После имплантации бластоцисты внутриклеточная энергия в основном поступает за счет гликолиза. Таким образом, очевидна важная роль митохондрий в обеспечении энергией эмбриогенеза. Коэнзим Q10 (CoQ10) является мощным эндогенным антиоксидантом, локализованным в мембранах, который защищает циркулирующие липопротеиды от перекисного окисления липидов. Результаты нескольких недавних клинических исследований показали, что экзогенные добавки CoQ10 обладают антиоксидантным действием и могут быть потенциальной терапией для снижения окислительного стресса. Дефицит CoQ10 увеличивает риск нарушения эмбрионального развития; однако эта взаимосвязь по-прежнему остается неясной. Учитывая, что на уровень CoQ10 влияют ферменты, участвующие в его синтезе, трудно сказать, вызваны ли нарушения дефицитом CoQ10 или являются прямым результатом дефектов в целевом гене. Было показано, что в отсутствие CoQ10 происходит снижение синтеза АТФ и усиление окислительного стресса в митохондриях – два биологических процесса, влияющих на эмбриональное развитие. В обзоре подчеркивается важность CoQ10 как антиоксиданта для улучшения качества яйцеклеток, а также показана его ключевая роль в эмбриональном развитии. Необходимо проведение дальнейших исследований, направленных на изучение метаболических изменений в процессе эмбриогенеза, а также механизма влияния CoQ10.

эмбриогенезкоэнзим Q10CoQ10окислительный стрессрепродукциямитохондрииорганогенезоогенезсперматогенезembryogenesiscoenzyme Q10CoQ10oxidative stressreproductionmitochondriaorganogenesisoogenesisspermatogenesisReferencesБашмакова Н.В., Третьякова Т.Б., Демченко Н.С. Цитогенетические нарушения у эмбриона при неразвивающейся беременности. Российский вестник акушера-гинеколога. 2013;13(4):18–21.Bashmakova N.V., Tret'iakova T.B., Demchenko N.S. Cytogenetic disorders in embryos during non-developing pregnancy. [Citogeneticheskie narusheniya u embriona pri nerazvivayushchejsya beremennosti]. Rossijskij vestnik akushera-ginekologa. 2013;13(4):18–21. (In Russ.).Новикова Н.Ю., Цибизова В.И., Первунина Т.М., Малушко А.В. Нутрициология и образ жизни при беременности. Российский журнал персонализированной медицины. 2023;3(2):82–92. https://doi.org/10.18705/2782-3806-2023-3-2-82-92.Novikova N.Yu., Tsibizova V.I., Pervunina T.M., Malushko A.V. Nutritionology and lifestyle during pregnancy. [Nutriciologiya i obraz zhizni pri beremennosti]. Rossijskij zhurnal personalizirovannoj mediciny. 2023;3(2):82–92. (In Russ.). https://doi.org/10.18705/2782-3806-2023-3-2-82-92.Святова Г.С., Березина Г.М., Муртазалиева А.В. Генетические аспекты идиопатической формы привычного невынашивания беременности. Медицинская генетика. 2020;19(11):83–4. https://doi.org/10.25557/2073-7998.2020.11.83-84.Svyatova G.S., Berezina G.M., Murtazaliyeva A.V. Genetic aspects of the idiopathic form of habitual miscarriage. [Geneticheskie aspekty idiopaticheskoj formy privychnogo nevynashivaniya beremennosti]. Medicinskaya genetika. 2020;19(11):83–4. (In Russ.). https://doi.org/10.25557/2073-7998.2020.11.83-84.Адамян Л.В., Геворгян А.П. Аутофагия как новое звено в механизме развития нарушений репродуктивной системы (обзор литературы). Проблемы репродукции. 2019;25(5):6–14.Adamyan L.V., Gevorgyan A.P. Autophagy as a new link in the mechanism of development of disorders of the reproductive system (literature review). [Autofagiya kak novoe zveno v mekhanizme razvitiya narushenij reproduktivnoj sistemy (obzor literatury)]. Problemy reprodukcii. 2019;25(5):6–14.Zhao J., Yao K., Yu H. et al. Metabolic remodelling during early mouse embryo development. Nat Metab. 2021;3(10):1372–84. https://doi.org/10.1038/s42255-021-00464-x.Zhao J., Yao K., Yu H. et al. Metabolic remodelling during early mouse embryo development. Nat Metab. 2021;3(10):1372–84. https://doi.org/10.1038/s42255-021-00464-x.Motiei M., Vaculikova K., Cela A. et al. Non-invasive human embryo metabolic assessment as a developmental criterion. J Clin Med. 2020;9(12):4094. https://doi.org/10.3390/jcm9124094.Motiei M., Vaculikova K., Cela A. et al. Non-invasive human embryo metabolic assessment as a developmental criterion. J Clin Med. 2020;9(12):4094. https://doi.org/10.3390/jcm9124094.Ayer A., Fazakerley D.J., Suarna C. et al. Genetic screening reveals phospholipid metabolism as a key regulator of the biosynthesis of the redox-active lipid coenzyme Q. Redox Biol. 2021;46:102127. https://doi.org/10.1016/j.redox.2021.102127.Ayer A., Fazakerley D.J., Suarna C. et al. Genetic screening reveals phospholipid metabolism as a key regulator of the biosynthesis of the redox-active lipid coenzyme Q. Redox Biol. 2021;46:102127. https://doi.org/10.1016/j.redox.2021.102127.You X., Ryu M.J., Cho E. et al. Embryonic expression of nrasG 12 D leads to embryonic lethality and cardiac defects. Front Cell Dev Biol. 2021;9:633661. https://doi.org/10.3389/fcell.2021.633661.You X., Ryu M.J., Cho E. et al. Embryonic Expression of nrasG 12 D leads to embryonic lethality and cardiac defects. Front Cell Dev Biol. 2021;9:633661. https://doi.org/10.3389/fcell.2021.633661.Wu Y., Yang D., Chen G.Y. Targeted disruption of Rab1a causes early embryonic lethality. Int J Mol Med. 2022;49(4):46. https://doi.org/10.3892/ijmm.2022.5101.Wu Y., Yang D., Chen G.Y. Targeted disruption of Rab1a causes early embryonic lethality. Int J Mol Med. 2022;49(4):46. https://doi.org/10.3892/ijmm.2022.5101.Liang R., Chen X., Zhang Y. et al. Clinical features and gene variation analysis of COQ8B nephropathy: Report of seven cases. Front Pediatr. 2023;10:1030191. https://doi.org/10.3389/fped.2022.1030191.Liang R., Chen X., Zhang Y. et al. Clinical features and gene variation analysis of COQ8B nephropathy: Report of seven cases. Front Pediatr. 2023;10:1030191. https://doi.org/10.3389/fped.2022.1030191.Somnay Y.R., Wang A., Griffiths K.K., Levy R.J. Altered brown adipose tissue mitochondrial function in newborn fragile X syndrome mice. Mitochondrion. 2022;65:1–10. https://doi.org/10.1016/j.mito.2022.04.003.Somnay Y.R., Wang A., Griffiths K.K., Levy R.J. Altered brown adipose tissue mitochondrial function in newborn fragile X syndrome mice. Mitochondrion. 2022;65:1–10. https://doi.org/10.1016/j.mito.2022.04.003.Guerra R.M., Pagliarini D.J. Coenzyme Q biochemistry and biosynthesis. Trends Biochem Sci. 2023;48(5):463–76. https://doi.org/10.1016/j.tibs.2022.12.006.Guerra R.M., Pagliarini D.J. Coenzyme Q biochemistry and biosynthesis. Trends Biochem Sci. 2023;48(5):463–76. https://doi.org/10.1016/j.tibs.2022.12.006.Gutierrez-Mariscal F.M., Arenas-de Larriva A.P., Limia-Perez L. et al. Coenzyme Q10 supplementation for the reduction of oxidative stress: clinical implications in the treatment of chronic diseases. Int J Mol Sci. 2020;21(21):7870. https://doi.org/10.3390/ijms21217870.Gutierrez-Mariscal F.M., Arenas-de Larriva A.P., Limia-Perez L. et al. Coenzyme Q10 supplementation for the reduction of oxidative stress: clinical implications in the treatment of chronic diseases. Int J Mol Sci. 2020;21(21):7870. https://doi.org/10.3390/ijms21217870.Navas P., Sanz A. Editorial: "Mitochondrial coenzyme Q homeostasis: Signalling, respiratory chain stability and diseases". Free Radic Biol Med. 2021;169:12–3. https://doi.org/10.1016/j.freeradbiomed.2021.04.005.Navas P., Sanz A. Editorial: "Mitochondrial coenzyme Q homeostasis: Signalling, respiratory chain stability and diseases". Free Radic Biol Med. 2021;169:12–3. https://doi.org/10.1016/j.freeradbiomed.2021.04.005.Alcázar-Fabra M., Rodríguez-Sánchez F., Trevisson E., Brea-Calvo G. Primary Coenzyme Q deficiencies: A literature review and online platform of clinical features to uncover genotype-phenotype correlations. Free Radic Biol Med. 2021;167:141–80. https://doi.org/10.1016/j.freeradbiomed.2021.02.046.Alcázar-Fabra M., Rodríguez-Sánchez F., Trevisson E., Brea-Calvo G. Primary Coenzyme Q deficiencies: A literature review and online platform of clinical features to uncover genotype-phenotype correlations. Free Radic Biol Med. 2021;167:141–80. https://doi.org/10.1016/j.freeradbiomed.2021.02.046.Zhao M., Tian Z., Zhao D. et al. L-shaped association between dietary coenzyme Q10 intake and high-sensitivity C-reactive protein in Chinese adults: a national cross-sectional study. Food Funct. 2023;14(21):9815–24. https://doi.org/10.1039/d3fo00978e.Zhao M., Tian Z., Zhao D. et al. L-shaped association between dietary coenzyme Q10 intake and high-sensitivity C-reactive protein in Chinese adults: a national cross-sectional study. Food Funct. 2023;14(21):9815–24. https://doi.org/10.1039/d3fo00978e.Громова О.А., Торшин И.Ю. Молекулярная фармакология коэнзима Q10 в контексте лечения гиперлипидемических состояний. ФАРМАКОЭКОНОМИКА. Современная фармакоэкономика и фармакоэпидемиология. 2023;16(2):345–57. https://doi.org/10.17749/2070-4909/farmakoekonomika.2023.186.Gromova O.A., Torshin I.Yu. Molecular pharmacology of coenzyme Q10 in the context of treatment of hyperlipidemic conditions. [Molekulyarnaya farmakologiya koenzima Q10 v kontekste lecheniya giperlipidemicheskih sostoyanij]. FARMAKOEKONOMIKA. Modern Pharmacoeconomics and Pharmacoepidemiology. 2023;16(2):345–57. (In Russ.). https://doi.org/10.17749/2070-4909/farmakoekonomika.2023.186.Lapuente-Brun E., Moreno-Loshuertos R., Acín-Pérez R. et al. Supercomplex assembly determines electron flux in the mitochondrial electron transport chain. Science. 2013;340(6140):1567–70. https://doi.org/10.1126/science.1230381.Lapuente-Brun E., Moreno-Loshuertos R., Acín-Pérez R. et al. Supercomplex assembly determines electron flux in the mitochondrial electron transport chain. Science. 2013;340(6140):1567–70. https://doi.org/10.1126/science.1230381.Griffiths K.K., Wang A., Levy R.J. Assessment of open probability of the mitochondrial permeability transition pore in the setting of coenzyme Q excess. J Vis Exp. 2022;(184). https://doi.org/10.3791/63646.Griffiths K.K., Wang A., Levy R.J. Assessment of open probability of the mitochondrial permeability transition pore in the setting of coenzyme Q excess. J Vis Exp. 2022;(184). https://doi.org/10.3791/63646.Oh J.N., Lee M., Choe G.C. et al. The number of primitive endoderm cells in the inner cell mass is regulated by platelet-derived growth factor signaling in porcine preimplantation embryos. Anim Biosci. 2023;36(8):1180–9. https://doi.org/10.5713/ab.22.0481.Oh J.N., Lee M., Choe G.C. et al. The number of primitive endoderm cells in the inner cell mass is regulated by platelet-derived growth factor signaling in porcine preimplantation embryos. Anim Biosci. 2023;36(8):1180–9. https://doi.org/10.5713/ab.22.0481.Gauster M., Moser G., Wernitznig S. et al. Early human trophoblast development: from morphology to function. Cell Mol Life Sci. 2022;79(6):345. https://doi.org/10.1007/s00018-022-04377-0.Gauster M., Moser G., Wernitznig S. et al. Early human trophoblast development: from morphology to function. Cell Mol Life Sci. 2022;79(6):345. https://doi.org/10.1007/s00018-022-04377-0.Wardle F.C. Mesoderm differentiation in vertebrate development and regenerative medicine. Semin Cell Dev Biol. 2022;127:1–2. https://doi.org/10.1016/j.semcdb.2022.02.014.Wardle F.C. Mesoderm differentiation in vertebrate development and regenerative medicine. Semin Cell Dev Biol. 2022;127:1–2. https://doi.org/10.1016/j.semcdb.2022.02.014.Adhikari D., Lee I.W., Yuen W.S., Carroll J. Oocyte mitochondria-key regulators of oocyte function and potential therapeutic targets for improving fertility. Biol Reprod. 2022;106(2):366–77. https://doi.org/10.1093/biolre/ioac024.Adhikari D., Lee I.W., Yuen W.S., Carroll J. Oocyte mitochondria-key regulators of oocyte function and potential therapeutic targets for improving fertility. Biol Reprod. 2022;106(2):366–77. https://doi.org/10.1093/biolre/ioac024.Непша О.С., Кулакова Е.В., Екимов А.Н. и др. Использование митохондриальной ДНК эмбрионов в качества предиктора эффективности программ вспомогательных репродуктивных технологий. Акушерство и гинекология. 2021;11:125–34. https://doi.org/10.18565/aig.2021.11.125-134.Nepsha O.S., Kulakova E.V., Ekimov A.N. et al. Value of embryonic mitochondrial DNA in predicting the effectiveness of assisted reproductive technologies. [Ispol'zovanie mitohondrial'noj DNK embrionov v kachestva prediktora effektivnosti programm vspomogatel'nyh reproduktivnyh tekhnologij]. Akusherstvo i ginekologiya. 2021;11:125–34. (In Russ.). https://doi.org/10.18565/aig.2021.11.125-134.Kang M.H., Kim Y.J., Lee J.H. Mitochondria in reproduction. Clin Exp Reprod Med. 2023;50(1):1–11. https://doi.org/10.5653/cerm.2022.05659.Kang M.H., Kim Y.J., Lee J.H. Mitochondria in reproduction. Clin Exp Reprod Med. 2023;50(1):1–11. https://doi.org/10.5653/cerm.2022.05659.Czernik M., Winiarczyk D., Sampino S. et al. Mitochondrial function and intracellular distribution is severely affected in in vitro cultured mouse embryos. Sci Rep. 2022;12(1):16152. https://doi.org/10.1038/s41598-022-20374-6.Czernik M., Winiarczyk D., Sampino S. et al. Mitochondrial function and intracellular distribution is severely affected in in vitro cultured mouse embryos. Sci Rep. 2022;12(1):16152. https://doi.org/10.1038/s41598-022-20374-6.Marchante M., Ramirez-Martin N., Buigues A. et al. Deciphering reproductive aging in women using a NOD/SCID mouse model for distinct physiological ovarian phenotypes. Aging (Albany NY). 2023;15(20):10856–74. https://doi.org/10.18632/aging.205086.Marchante M., Ramirez-Martin N., Buigues A. et al. Deciphering reproductive aging in women using a NOD/SCID mouse model for distinct physiological ovarian phenotypes. Aging (Albany NY). 2023;15(20):10856–74. https://doi.org/10.18632/aging.205086.Адамян Л.В., Сибирская Е.В., Щерина А.В. Патогенетические аспекты преждевременной недостаточности яичников. Проблемы репродукции. 2021;27(1):6–12.Adamyan L.V., Sibirskaya E.V., Shcherina A.V. Pathogenetic aspects of premature ovarian failure. [Patogeneticheskie aspekty prezhdevremennoj nedostatochnosti yaichnikov]. Problemy reprodukcii. 2021;27(1):6–12. (In Russ.).van der Reest J., Nardini Cecchino G., Haigis M.C., Kordowitzki P. Mitochondria: Their relevance during oocyte ageing. Ageing Res Rev. 2021;70:101378. https://doi.org/10.1016/j.arr.2021.101378.van der Reest J., Nardini Cecchino G., Haigis M.C., Kordowitzki P. Mitochondria: Their relevance during oocyte ageing. Ageing Res Rev. 2021;70:101378. https://doi.org/10.1016/j.arr.2021.101378.Jiang Z., Shen H. Mitochondria: emerging therapeutic strategies for oocyte rescue. Reprod Sci. 2022;29(3):711–22. https://doi.org/10.1007/s43032-021-00523-4.Jiang Z., Shen H. Mitochondria: emerging therapeutic strategies for oocyte rescue. Reprod Sci. 2022;29(3):711–22. https://doi.org/10.1007/s43032-021-00523-4.Hudson G., Takeda Y., Herbert M. Reversion after replacement of mitochondrial DNA. Nature. 2019;574(7778):8–11. https://doi.org/10.1038/s41586-019-1623-3.Hudson G., Takeda Y., Herbert M. Reversion after replacement of mitochondrial DNA. Nature. 2019;574(7778):8–11. https://doi.org/10.1038/s41586-019-1623-3.Блашкив Т.В., Шепель А.А.. Вознесенская Т.Ю. Экспрессия генов клетками кумулюсного окружения ооцита в период овуляции и оплодотворения (обзор литературы). Проблемы репродукции. 2014;(1):55–8.Blashkiv T.V., Shepel' A.A., Voznesenskaia T.Iu. Review of the cumulus cell gene expression during ovulation and fertilization. [Ekspressiya genov kletkami kumulyusnogo okruzheniya oocita v period ovulyacii i oplodotvoreniya (obzor literatury)]. Problemy reprodukcii. 2014;(1):55–8.Hu Y., Zhang R., Zhang S. et al. Transcriptomic profiles reveal the characteristics of oocytes and cumulus cells at GV, MI, and MII in follicles before ovulation. J Ovarian Res. 2023;16(1):225. https://doi.org/10.1186/s13048-023-01291-2.Hu Y., Zhang R., Zhang S. et al. Transcriptomic profiles reveal the characteristics of oocytes and cumulus cells at GV, MI, and MII in follicles before ovulation. J Ovarian Res. 2023;16(1):225. https://doi.org/10.1186/s13048-023-01291-2.Babayev E., Duncan F.E. Age-associated changes in cumulus cells and follicular fluid: the local oocyte microenvironment as a determinant of gamete quality. Biol Reprod. 2022;106(2):351–65. https://doi.org/10.1093/biolre/ioab241.Babayev E., Duncan F.E. Age-associated changes in cumulus cells and follicular fluid: the local oocyte microenvironment as a determinant of gamete quality. Biol Reprod. 2022;106(2):351–65. https://doi.org/10.1093/biolre/ioab241.Krawczyk K., Marynowicz W., Pich K. et al. Persistent organic pollutants affect steroidogenic and apoptotic activities in granulosa cells and reactive oxygen species concentrations in oocytes in the mouse. Reprod Fertil Dev. 2023;35(3):294–305. https://doi.org/10.1071/RD21326.Krawczyk K., Marynowicz W., Pich K. et al. Persistent organic pollutants affect steroidogenic and apoptotic activities in granulosa cells and reactive oxygen species concentrations in oocytes in the mouse. Reprod Fertil Dev. 2023;35(3):294–305. https://doi.org/10.1071/RD21326.Yu L., Liu M., Xu S. et al. Follicular fluid steroid and gonadotropic hormone levels and mitochondrial function from exosomes predict embryonic development. Front Endocrinol (Lausanne). 2022;13:1025523. https://doi.org/10.3389/fendo.2022.1025523.Yu L., Liu M., Xu S. et al. Follicular fluid steroid and gonadotropic hormone levels and mitochondrial function from exosomes predict embryonic development. Front Endocrinol (Lausanne). 2022;13:1025523. https://doi.org/10.3389/fendo.2022.1025523.Gasmi A., Bjørklund G., Mujawdiya P.K. et al. Coenzyme Q10 in aging and disease. Crit Rev Food Sci Nutr. 2024;64(12):3907–19. https://doi.org/10.1080/10408398.2022.2137724.Gasmi A., Bjørklund G., Mujawdiya P.K. et al. Coenzyme Q10 in aging and disease. Crit Rev Food Sci Nutr. 2024;64(12):3907–19. https://doi.org/10.1080/10408398.2022.2137724.Yang C.X., Liu S., Miao J.K. et al. CoQ10 improves meiotic maturation of pig oocytes through enhancing mitochondrial function and suppressing oxidative stress. Theriogenology. 2021;159:77–86. https://doi.org/10.1016/j.theriogenology.2020.10.009.Yang C.X., Liu S., Miao J.K. et al. CoQ10 improves meiotic maturation of pig oocytes through enhancing mitochondrial function and suppressing oxidative stress. Theriogenology. 2021;159:77–86. https://doi.org/10.1016/j.theriogenology.2020.10.009.Brown A.M., McCarthy H.E. The Effect of CoQ10 supplementation on ART treatment and oocyte quality in older women. Hum Fertil (Camb). 2023;26(6):1544–52. https://doi.org/10.1080/14647273.2023.2194554.Brown A.M., McCarthy H.E. The Effect of CoQ10 supplementation on ART treatment and oocyte quality in older women. Hum Fertil (Camb). 2023;26(6):1544–52. https://doi.org/10.1080/14647273.2023.2194554.Yang J., Feng T., Li S. et al. Human follicular fluid shows diverse metabolic profiles at different follicle developmental stages. Reprod Biol Endocrinol. 2020;18(1):74. https://doi.org/10.1186/s12958-020-00631-x.Yang J., Feng T., Li S. et al. Human follicular fluid shows diverse metabolic profiles at different follicle developmental stages. Reprod Biol Endocrinol. 2020;18(1):74. https://doi.org/10.1186/s12958-020-00631-x.Giannubilo S.R., Orlando P., Silvestri S. et al. CoQ10 Supplementation in patients undergoing IVF-ET: The relationship with follicular fluid content and oocyte maturity. Antioxidants (Basel). 2018;7(10):141. https://doi.org/10.3390/antiox7100141.Giannubilo S.R., Orlando P., Silvestri S. et al. CoQ10 Supplementation in patients undergoing IVF-ET: The relationship with follicular fluid content and oocyte maturity. Antioxidants (Basel). 2018;7(10):141. https://doi.org/10.3390/antiox7100141.Lee C.H., Kang M.K., Sohn D.H. et al. Coenzyme Q10 ameliorates the quality of mouse oocytes during in vitro culture. Zygote. 2022;30(2):249–57. https://doi.org/10.1017/S0967199421000617.Lee C.H., Kang M.K., Sohn D.H. et al. Coenzyme Q10 ameliorates the quality of mouse oocytes during in vitro culture. Zygote. 2022;30(2):249–57. https://doi.org/10.1017/S0967199421000617.Yang L., Wang H., Song S. et al. Systematic understanding of anti-aging effect of coenzyme Q10 on oocyte through a network pharmacology approach. Front Endocrinol (Lausanne). 2022;13:813772. https://doi.org/10.3389/fendo.2022.813772.Yang L., Wang H., Song S. et al. Systematic understanding of anti-aging effect of coenzyme Q10 on oocyte through a network pharmacology approach. Front Endocrinol (Lausanne). 2022;13:813772. https://doi.org/10.3389/fendo.2022.813772 .Heydarnejad A., Ostadhosseini S., Varnosfaderani S.R. et al. Supplementation of maturation medium with CoQ10 enhances developmental competence of ovine oocytes through improvement of mitochondrial function. Mol Reprod Dev. 2019;86(7):812–24. https://doi.org/10.1002/mrd.23159.Heydarnejad A., Ostadhosseini S., Varnosfaderani S.R. et al. Supplementation of maturation medium with CoQ10 enhances developmental competence of ovine oocytes through improvement of mitochondrial function. Mol Reprod Dev. 2019;86(7):812–24. https://doi.org/10.1002/mrd.23159.Ruiz-Conca M., Gardela J., Mogas T. et al. Apoptosis and glucocorticoid-related genes mRNA expression is modulated by coenzyme Q10 supplementation during in vitro maturation and vitrification of bovine oocytes and cumulus cells. Theriogenology. 2022;192:62–72. https://doi.org/10.1016/j.theriogenology.2022.08.030.Ruiz-Conca M., Gardela J., Mogas T. et al. Apoptosis and glucocorticoid-related genes mRNA expression is modulated by coenzyme Q10 supplementation during in vitro maturation and vitrification of bovine oocytes and cumulus cells. Theriogenology. 2022;192:62–72. https://doi.org/10.1016/j.theriogenology.2022.08.030.Gendelman M., Roth Z. Incorporation of coenzyme Q10 into bovine oocytes improves mitochondrial features and alleviates the effects of summer thermal stress on developmental competence. Biol Reprod. 2012;87(5):118. https://doi.org/10.1095/biolreprod.112.101881.Gendelman M., Roth Z. Incorporation of coenzyme Q10 into bovine oocytes improves mitochondrial features and alleviates the effects of summer thermal stress on developmental competence. Biol Reprod. 2012;87(5):118. https://doi.org/10.1095/biolreprod.112.101881.Miao Y., Cui Z., Gao Q. et al. Nicotinamide mononucleotide supplementation reverses the declining quality of maternally aged oocytes. Cell Rep. 2020;32(5):107987. https://doi.org/10.1016/j.celrep.2020.107987.Miao Y., Cui Z., Gao Q. et al. Nicotinamide mononucleotide supplementation reverses the declining quality of maternally aged oocytes. Cell Rep. 2020;32(5):107987. https://doi.org/10.1016/j.celrep.2020.107987.Nikalayevich E., Terret M.E. Meiosis: Actin and microtubule networks drive chromosome clustering in oocytes. Curr Biol. 2023;33(7):272–4. https://doi.org/10.1016/j.cub.2023.02.061.Nikalayevich E., Terret M.E. Meiosis: Actin and microtubule networks drive chromosome clustering in oocytes. Curr Biol. 2023;33(7):272–4. https://doi.org/10.1016/j.cub.2023.02.061.Miao Y., Zhou C., Cui Z. et al. Postovulatory aging causes the deterioration of porcine oocytes via induction of oxidative stress. FASEB J. 2018;32(3):1328–37. https://doi.org/10.1096/fj.201700908R.Miao Y., Zhou C., Cui Z. et al. Postovulatory aging causes the deterioration of porcine oocytes via induction of oxidative stress. FASEB J. 2018;32(3):1328–37. https://doi.org/10.1096/fj.201700908R.Zhang M., Shi Yang X., Zhang Y. et al. Coenzyme Q10 ameliorates the quality of postovulatory aged oocytes by suppressing DNA damage and apoptosis. Free Radic Biol Med. 2019;143:84–94. https://doi.org/10.1016/j.freeradbiomed.2019.08.002.Zhang M., Shi Yang X., Zhang Y. et al. Coenzyme Q10 ameliorates the quality of postovulatory aged oocytes by suppressing DNA damage and apoptosis. Free Radic Biol Med. 2019;143:84–94. https://doi.org/10.1016/j.freeradbiomed.2019.08.002.Shaw E., Talwadekar M., Rashida Z. et al. Anabolic SIRT4 exerts retrograde control over TORC1 signaling by glutamine sparing in the mitochondria. Mol Cell Biol. 2020;40(2):e00212–19. https://doi.org/10.1128/MCB.00212-19.Shaw E., Talwadekar M., Rashida Z. et al. Anabolic SIRT4 exerts retrograde control over TORC1 signaling by glutamine sparing in the mitochondria. Mol Cell Biol. 2020;40(2):e00212–19. https://doi.org/10.1128/MCB.00212-19.He L., Liu Q., Cheng J. et al. SIRT4 in ageing. Biogerontology. 2023;24(3):347–62. https://doi.org/10.1007/s10522-023-10022-5.He L., Liu Q., Cheng J. et al. SIRT4 in ageing. Biogerontology. 2023;24(3):347–62. https://doi.org/10.1007/s10522-023-10022-5.Xing X., Zhang J., Zhang J. et al. Coenzyme Q10 supplement rescues postovulatory oocyte aging by regulating SIRT4 expression. Curr Mol Pharmacol. 2022;15(1):190–203. https://doi.org/10.2174/1874467214666210420112819.Xing X., Zhang J., Zhang J. et al. Coenzyme Q10 supplement rescues postovulatory oocyte aging by regulating SIRT4 expression. Curr Mol Pharmacol. 2022;15(1):190–203. https://doi.org/10.2174/1874467214666210420112819.Ben-Meir A., Burstein E., Borrego-Alvarez A. et al. Coenzyme Q10 restores oocyte mitochondrial function and fertility during reproductive aging. Aging Cell. 2015;14(5):887–95. https://doi.org/10.1111/acel.12368.Ben-Meir A., Burstein E., Borrego-Alvarez A. et al. Coenzyme Q10 restores oocyte mitochondrial function and fertility during reproductive aging. Aging Cell. 2015;14(5):887–95. https://doi.org/10.1111/acel.12368.Del Bianco D., Gentile R., Sallicandro L. et al. Electro-metabolic coupling of cumulus-oocyte complex. Int J Mol Sci. 2024;25(10):5349. https://doi.org/10.3390/ijms25105349.Del Bianco D., Gentile R., Sallicandro L. et al. Electro-metabolic coupling of cumulus-oocyte complex. Int J Mol Sci. 2024;25(10):5349. https://doi.org/10.3390/ijms25105349.Ben-Meir A., Kim K., McQuaid R. et al. Co-enzyme Q10 supplementation rescues cumulus cells dysfunction in a maternal aging model. Antioxidants (Basel). 2019;8(3):58. https://doi.org/10.3390/antiox8030058.Ben-Meir A., Kim K., McQuaid R. et al. Co-enzyme Q10 supplementation rescues cumulus cells dysfunction in a maternal aging model. Antioxidants (Basel). 2019;8(3):58. https://doi.org/10.3390/antiox8030058.Bellusci M., García-Silva M.T., Martínez de Aragón A., Martín M.A. Distal phalangeal erythema in an infant with biallelic PDSS1 mutations: expanding the phenotype of primary Coenzyme Q10 deficiency. JIMD Rep. 2021;62(1):3–5. https://doi.org/10.1002/jmd2.12216.Bellusci M., García-Silva M.T., Martínez de Aragón A., Martín M.A. Distal phalangeal erythema in an infant with biallelic PDSS1 mutations: expanding the phenotype of primary Coenzyme Q10 deficiency. JIMD Rep. 2021;62(1):3–5. https://doi.org/10.1002/jmd2.12216.Li M., Yue Z., Lin H. et al. COQ2 mutation associated isolated nephropathy in two siblings from a Chinese pedigree. Ren Fail. 2021;43(1):97–101. https://doi.org/10.1080/0886022X.2020.1864402.Li M., Yue Z., Lin H. et al. COQ2 mutation associated isolated nephropathy in two siblings from a Chinese pedigree. Ren Fail. 2021;43(1):97–101. https://doi.org/10.1080/0886022X.2020.1864402.Laugwitz L., Seibt A., Herebian D. et al. Human COQ4 deficiency: delineating the clinical, metabolic and neuroimaging phenotypes. J Med Genet. 2022;59(9):878–87. https://doi.org/10.1136/jmedgenet-2021-107729.Laugwitz L., Seibt A., Herebian D. et al. Human COQ4 deficiency: delineating the clinical, metabolic and neuroimaging phenotypes. J Med Genet. 2022;59(9):878–87. https://doi.org/10.1136/jmedgenet-2021-107729.Wang N., Zheng Y., Zhang L. et al. A family segregating lethal primary coenzyme Q10 deficiency due to two novel COQ6 variants. Front Genet. 2022;12:811833. https://doi.org/10.3389/fgene.2021.811833.Wang N., Zheng Y., Zhang L. et al. A family segregating lethal primary coenzyme Q10 deficiency due to two novel COQ6 variants. Front Genet. 2022;12:811833. https://doi.org/10.3389/fgene.2021.811833.Olgac A., Öztoprak Ü., Kasapkara C.S. et al. A rare case of primary coenzyme Q10 deficiency due to COQ9 mutation. J Pediatr Endocrinol Metab. 2020;33(1):165–70. https://doi.org/10.1515/jpem-2019-0245.Olgac A., Öztoprak Ü., Kasapkara C.S. et al. A rare case of primary coenzyme Q10 deficiency due to COQ9 mutation. J Pediatr Endocrinol Metab. 2020;33(1):165–70. https://doi.org/10.1515/jpem-2019-0245.Howden S.E., Vanslambrouck J.M., Wilson S.B. et al. Reporter-based fate mapping in human kidney organoids confirms nephron lineage relationships and reveals synchronous nephron formation. EMBO Rep. 2019;20(4):e47483. https://doi.org/10.15252/embr.201847483.Howden S.E., Vanslambrouck J.M., Wilson S.B. et al. Reporter-based fate mapping in human kidney organoids confirms nephron lineage relationships and reveals synchronous nephron formation. EMBO Rep. 2019;20(4):e47483. https://doi.org/10.15252/embr.201847483.Drovandi S., Lipska-Ziętkiewicz B.S., Ozaltin F. et al.; PodoNet Consortium; mitoNET Consortium; CCGKDD Consortium; Schaefer F. Variation of the clinical spectrum and genotype-phenotype associations in Coenzyme Q10 deficiency associated glomerulopathy. Kidney Int. 2022;102(3):592–603. https://doi.org/10.1016/j.kint.2022.02.040.Drovandi S., Lipska-Ziętkiewicz B.S., Ozaltin F. et al.; PodoNet Consortium; mitoNET Consortium; CCGKDD Consortium; Schaefer F. Variation of the clinical spectrum and genotype-phenotype associations in Coenzyme Q10 deficiency associated glomerulopathy. Kidney Int. 2022;102(3):592–603. https://doi.org/10.1016/j.kint.2022.02.040.Zhai S.B., Zhang L., Sun B.C. et al. Early-onset COQ8B (ADCK4) glomerulopathy in a child with isolated proteinuria: a case report and literature review. BMC Nephrol. 2020;21(1):406. https://doi.org/10.1186/s12882-020-02038-7.Zhai S.B., Zhang L., Sun B.C. et al. Early-onset COQ8B (ADCK4) glomerulopathy in a child with isolated proteinuria: a case report and literature review. BMC Nephrol. 2020;21(1):406. https://doi.org/10.1186/s12882-020-02038-7.Stańczyk M., Bałasz-Chmielewska I., Lipska-Ziętkiewicz B., Tkaczyk M. CoQ10-related sustained remission of proteinuria in a child with COQ6 glomerulopathy – a case report. Pediatr Nephrol. 2018;33(12):2383–7. https://doi.org/10.1007/s00467-018-4083-3.Stańczyk M., Bałasz-Chmielewska I., Lipska-Ziętkiewicz B., Tkaczyk M. CoQ10-related sustained remission of proteinuria in a child with COQ6 glomerulopathy – a case report. Pediatr Nephrol. 2018;33(12):2383–7. https://doi.org/10.1007/s00467-018-4083-3.Suciu S.K., Caspary T. Cilia, neural development and disease. Semin Cell Dev Biol. 2021;110:34–42. https://doi.org/10.1016/j.semcdb.2020.07.014.Suciu S.K., Caspary T. Cilia, neural development and disease. Semin Cell Dev Biol. 2021;110:34–42. https://doi.org/10.1016/j.semcdb.2020.07.014.Zoghbi J.F., Licznerski P., Yang M. et al. Inefficient thermogenic mitochondrial respiration due to futile proton leak in a mouse model of fragile X syndrome. FASEB J. 2020;34(6):7404–26. https://doi.org/10.1096/fj.202000283RR.Zoghbi J.F., Licznerski P., Yang M. et al. Inefficient thermogenic mitochondrial respiration due to futile proton leak in a mouse model of fragile X syndrome. FASEB J. 2020;34(6):7404–26. https://doi.org/10.1096/fj.202000283RR.Muigg V., Maier M.I., Kuenzli E., Neumayr A. Delayed cerebellar ataxia, a rare post-malaria neurological complication: Case report and review of the literature. Travel Med Infect Dis. 2021;44:102177. https://doi.org/10.1016/j.tmaid.2021.102177.Muigg V., Maier M.I., Kuenzli E., Neumayr A. Delayed cerebellar ataxia, a rare post-malaria neurological complication: Case report and review of the literature. Travel Med Infect Dis. 2021;44:102177. https://doi.org/10.1016/j.tmaid.2021.102177.Monfrini E., Pesini A., Biella F. et al. Whole-exome sequencing study of fibroblasts derived from patients with cerebellar ataxia referred to investigate CoQ10 deficiency. Neurol Genet. 2023;9(2):e200058. https://doi.org/10.1212/NXG.0000000000200058.Monfrini E., Pesini A., Biella F. et al. Whole-exome sequencing study of fibroblasts derived from patients with cerebellar ataxia referred to investigate CoQ10 deficiency. Neurol Genet. 2023;9(2):e200058. https://doi.org/10.1212/NXG.0000000000200058.Rius R., Bennett N.K., Bhattacharya K. et al. Biallelic pathogenic variants in COX11 are associated with an infantile-onset mitochondrial encephalopathy. Hum Mutat. 2022;43(12):1970–8. https://doi.org/10.1002/humu.24453.Rius R., Bennett N.K., Bhattacharya K. et al. Biallelic pathogenic variants in COX11 are associated with an infantile-onset mitochondrial encephalopathy. Hum Mutat. 2022;43(12):1970–8. https://doi.org/10.1002/humu.24453.Justine Perrin R., Rousset-Rouvière C., Garaix F. et al. COQ6 mutation in patients with nephrotic syndrome, sensorineural deafness, and optic atrophy. JIMD Rep. 2020;54(1):37–44. https://doi.org/10.1002/jmd2.12068.Justine Perrin R., Rousset-Rouvière C., Garaix F. et al. COQ6 mutation in patients with nephrotic syndrome, sensorineural deafness, and optic atrophy. JIMD Rep. 2020;54(1):37–44. https://doi.org/10.1002/jmd2.12068.Turnis M.E., Kaminska E., Smith K.H. et al. Requirement for antiapoptotic MCL-1 during early erythropoiesis. Blood. 2021;137(14):1945–58. https://doi.org/10.1182/blood.2020006916.Turnis M.E., Kaminska E., Smith K.H. et al. Requirement for antiapoptotic MCL-1 during early erythropoiesis. Blood. 2021;137(14):1945–58. https://doi.org/10.1182/blood.2020006916.Martinez P.A., Li R., Ramanathan H.N. et al. Smad2/3-pathway ligand trap luspatercept enhances erythroid differentiation in murine β-thalassaemia by increasing GATA-1 availability. J Cell Mol Med. 2020;24(11):6162–77. https://doi.org/10.1111/jcmm.15243.Martinez P.A., Li R., Ramanathan H.N. et al. Smad2/3-pathway ligand trap luspatercept enhances erythroid differentiation in murine β-thalassaemia by increasing GATA-1 availability. J Cell Mol Med. 2020;24(11):6162–77. https://doi.org/10.1111/jcmm.15243.Martell D.J., Merens H.E., Caulier A. et al. RNA polymerase II pausing temporally coordinates cell cycle progression and erythroid differentiation. Dev Cell. 2023;58(20):2112–2127.e4. https://doi.org/10.1016/j.devcel.2023.07.018.Martell D.J., Merens H.E., Caulier A. et al. RNA polymerase II pausing temporally coordinates cell cycle progression and erythroid differentiation. Dev Cell. 2023;58(20):2112–2127.e4. https://doi.org/10.1016/j.devcel.2023.07.018.Rossmann M.P., Hoi K., Chan V. et al. Cell-specific transcriptional control of mitochondrial metabolism by TIF1γ drives erythropoiesis. Science. 2021;372(6543):716–21. https://doi.org/10.1126/science.aaz2740.Rossmann M.P., Hoi K., Chan V. et al. Cell-specific transcriptional control of mitochondrial metabolism by TIF1γ drives erythropoiesis. Science. 2021;372(6543):716–21. https://doi.org/10.1126/science.aaz2740.Drakhlis L., Biswanath S., Farr C.M. et al. Human heart-forming organoids recapitulate early heart and foregut development. Nat Biotechnol. 2021;39(6):737–46. https://doi.org/10.1038/s41587-021-00815-9.Drakhlis L., Biswanath S., Farr C.M. et al. Human heart-forming organoids recapitulate early heart and foregut development. Nat Biotechnol. 2021;39(6):737–46. https://doi.org/10.1038/s41587-021-00815-9.Yan A., Liu Z., Song L. et al. Idebenone Alleviates neuroinflammation and modulates microglial polarization in LPS-stimulated BV2 cells and MPTP-induced Parkinson's disease mice. Front Cell Neurosci. 2019;12:529. https://doi.org/10.3389/fncel.2018.00529.Yan A., Liu Z., Song L. et al. Idebenone Alleviates neuroinflammation and modulates microglial polarization in LPS-stimulated BV2 cells and MPTP-induced Parkinson's disease mice. Front Cell Neurosci. 2019;12:529. https://doi.org/10.3389/fncel.2018.00529.Robichaux D.J., Harata M., Murphy E., Karch J. Mitochondrial permeability transition pore-dependent necrosis. J Mol Cell Cardiol. 2023;174:47–55. https://doi.org/10.1016/j.yjmcc.2022.11.003.Robichaux D.J., Harata M., Murphy E., Karch J. Mitochondrial permeability transition pore-dependent necrosis. J Mol Cell Cardiol. 2023;174:47–55. https://doi.org/10.1016/j.yjmcc.2022.11.003.Barajas M., Wang A., Griffiths K.K. et al. The newborn Fmr1 knockout mouse: a novel model of excess ubiquinone and closed mitochondrial permeability transition pore in the developing heart. Pediatr Res. 2021;89(3):456–63. https://doi.org/10.1038/s41390-020-1064-6.Barajas M., Wang A., Griffiths K.K. et al. The newborn Fmr1 knockout mouse: a novel model of excess ubiquinone and closed mitochondrial permeability transition pore in the developing heart. Pediatr Res. 2021;89(3):456–63. https://doi.org/10.1038/s41390-020-1064-6.Wang Y., Hekimi S. The CoQ biosynthetic di-iron carboxylate hydroxylase COQ7 is inhibited by in vivo metalation with manganese but remains functional by metalation with cobalt. MicroPubl Biol. 2022;2022:10.17912/micropub.biology.000635. https://doi.org/10.17912/micropub.biology.000635.Wang Y., Hekimi S. The CoQ biosynthetic di-iron carboxylate hydroxylase COQ7 is inhibited by in vivo metalation with manganese but remains functional by metalation with cobalt. MicroPubl Biol. 2022;2022:10.17912/micropub.biology.000635. https://doi.org/10.17912/micropub.biology.000635.Smith A.C., Ito Y., Ahmed A. et al.; Care4Rare Canada Consortium. A family segregating lethal neonatal coenzyme Q10 deficiency caused by mutations in COQ9. J Inherit Metab Dis. 2018;41(4):719–29. https://doi.org/10.1007/s10545-017-0122-7.Smith A.C., Ito Y., Ahmed A. et al.; Care4Rare Canada Consortium. A family segregating lethal neonatal coenzyme Q10 deficiency caused by mutations in COQ9. J Inherit Metab Dis. 2018;41(4):719–29. https://doi.org/10.1007/s10545-017-0122-7.Danhauser K., Herebian D., Haack T.B. et al. Fatal neonatal encephalopathy and lactic acidosis caused by a homozygous loss-of-function variant in COQ9. Eur J Hum Genet. 2016;24(3):450–4. https://doi.org/10.1038/ejhg.2015.133.Danhauser K., Herebian D., Haack T.B. et al. Fatal neonatal encephalopathy and lactic acidosis caused by a homozygous loss-of-function variant in COQ9. Eur J Hum Genet. 2016;24(3):450–4. https://doi.org/10.1038/ejhg.2015.133.Teran E., Hernández I., Tana L. et al. Mitochondria and coenzyme Q10 in the pathogenesis of preeclampsia. Front Physiol. 2018;9:1561. https://doi.org/10.3389/fphys.2018.01561.Teran E., Hernández I., Tana L. et al. Mitochondria and coenzyme Q10 in the pathogenesis of preeclampsia. Front Physiol. 2018;9:1561. https://doi.org/10.3389/fphys.2018.01561.Budani M.C., Tiboni G.M. Effects of supplementation with natural antioxidants on oocytes and preimplantation embryos. Antioxidants (Basel). 2020;9(7):612. https://doi.org/10.3390/antiox9070612.Budani M.C., Tiboni G.M. Effects of supplementation with natural antioxidants on oocytes and preimplantation embryos. Antioxidants (Basel). 2020;9(7):612. https://doi.org/10.3390/antiox9070612.

The authors declare that there are no conflicts of interest present.