RT Journal Article T1 Metabolic Targets of Coenzyme Q10 in Mitochondria. A1 Hidalgo-Gutierrez, Agustin A1 Gonzalez-Garcia, Pilar A1 Diaz-Casado, Maria Elena A1 Barriocanal-Casado, Eliana A1 Lopez-Herrador, Sergio A1 Quinzii, Catarina M A1 Lopez, Luis C K1 OxPhos K1 coenzyme Q10 K1 mitochondria K1 one-carbon metabolism K1 proline metabolism K1 sulfide metabolism K1 super-complexes K1 ubiquinol-10 K1 ubiquinone-10 AB Coenzyme Q10 (CoQ10) is classically viewed as an important endogenous antioxidant and key component of the mitochondrial respiratory chain. For this second function, CoQ molecules seem to be dynamically segmented in a pool attached and engulfed by the super-complexes I + III, and a free pool available for complex II or any other mitochondrial enzyme that uses CoQ as a cofactor. This CoQ-free pool is, therefore, used by enzymes that link the mitochondrial respiratory chain to other pathways, such as the pyrimidine de novo biosynthesis, fatty acid β-oxidation and amino acid catabolism, glycine metabolism, proline, glyoxylate and arginine metabolism, and sulfide oxidation metabolism. Some of these mitochondrial pathways are also connected to metabolic pathways in other compartments of the cell and, consequently, CoQ could indirectly modulate metabolic pathways located outside the mitochondria. Thus, we review the most relevant findings in all these metabolic functions of CoQ and their relations with the pathomechanisms of some metabolic diseases, highlighting some future perspectives and potential therapeutic implications. PB MDPI AG SN 2076-3921 YR 2021 FD 2021-03-23 LK http://hdl.handle.net/10668/17525 UL http://hdl.handle.net/10668/17525 LA en NO Hidalgo-Gutiérrez A, González-García P, Díaz-Casado ME, Barriocanal-Casado E, López-Herrador S, Quinzii C, et al. Metabolic Targets of Coenzyme Q10 in Mitochondria. Antioxidants (Basel). 2021 Mar 26;10(4):520. NO his work was supported by grants from Ministerio de Ciencia e Innovación, Spain, and the ERDF (RTI2018-093503-B-100), the Muscular Dystrophy Association (MDA-602322). C.M.Q. is supported by the Department of Defense (DOD) grant PR190511. A.H.-G. and P.G.-G. are ‘FPU fellows’ from the Ministerio de Universidades, Spain. S.L.-H. is supported by the “becas de colaboración” from the Ministerio de Universidades, Spain. E.B.-C. is supported by the Consejería de Salud, Junta de Andalucía, Spain. DS RISalud RD Apr 12, 2025