Metabolism and Functions of Coenzyme Q

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Pesini, A., Barriocanal-Casado, E., Compagnoni, G. M., Hidalgo-Gutierrez, A., Yanez, G., Bakkali, M., Chhonker, Y. S., Kleiner, G., Larrea, D., Tadesse, S., Lopez, L. C., Murry, D. J., Di Fonzo, A., Area-Gomez, E., & Quinzii, C. M. (2025). Coenzyme Q10 deficiency disrupts lipid metabolism by altering cholesterol homeostasis in neurons. Free Radical Biology and Medicine, 229, 441–457. https://doi.org/10.1016/j.freeradbiomed.2025.01.009
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Díaz-Casado, M. E., González-García, P., López-Herrador, S., Hidalgo-Gutiérrez, A., Jiménez-Sánchez, L., Barriocanal-Casado, E., Bakkali, M., van de Lest, C. H. A., Corral-Sarasa, J., Zaal, E. A., Berkers, C. R., & López, L. C. (2024). Oral β-RA induces metabolic rewiring leading to the rescue of diet-induced obesity. Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, 1870(7), 167283. https://doi.org/10.1016/j.bbadis.2024.167283
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Corral-Sarasa, J., Martínez-Gálvez, J. M., González-García, P., Wendling, O., Jiménez-Sánchez, L., López-Herrador, S., Quinzii, C. M., Díaz-Casado, M. E., & López, L. C. (2024). 4-Hydroxybenzoic acid rescues multisystemic disease and perinatal lethality in a mouse model of mitochondrial disease. Cell Reports, 43(5), 114148. https://doi.org/10.1016/j.celrep.2024.114148
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Monfrini, E., Pesini, A., Biella, F., Sobreira, C. F. R., Emmanuele, V., Brescia, G., Lopez, L. C., Tadesse, S., Hirano, M., Comi, G. P., Quinzii, C. M., & Di Fonzo, A. (2023). Whole-Exome Sequencing Study of Fibroblasts Derived From Patients With Cerebellar Ataxia Referred to Investigate CoQ10 Deficiency. Neurology Genetics, 9(2). https://doi.org/10.1212/nxg.0000000000200058
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