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β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9R239X mice.
Hidalgo-Gutiérrez A, Barriocanal-Casado E, Bakkali M, Díaz-Casado ME, Sánchez-Maldonado L, Romero M, Sayed RK, Prehn C, Escames G, Duarte J, Acuña-Castroviejo D, López LC. Hidalgo-Gutiérrez A, et al. EMBO Mol Med. 2019 Jan;11(1):e9466. doi: 10.15252/emmm.201809466. EMBO Mol Med. 2019. PMID: 30482867 Free PMC article.
CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome.
Luna-Sánchez M, Hidalgo-Gutiérrez A, Hildebrandt TM, Chaves-Serrano J, Barriocanal-Casado E, Santos-Fandila Á, Romero M, Sayed RK, Duarte J, Prokisch H, Schuelke M, Distelmaier F, Escames G, Acuña-Castroviejo D, López LC. Luna-Sánchez M, et al. EMBO Mol Med. 2017 Jan;9(1):78-95. doi: 10.15252/emmm.201606345. EMBO Mol Med. 2017. PMID: 27856619 Free PMC article.
In Vivo Determination of Mitochondrial Respiration in 1-Methyl-4-Phenyl-1,2,3,6-Tetrahydropyridine-Treated Zebrafish Reveals the Efficacy of Melatonin in Restoring Mitochondrial Normalcy.
Díaz-Casado ME, Rusanova I, Aranda P, Fernández-Ortiz M, Sayed RKA, Fernández-Gil BI, Hidalgo-Gutiérrez A, Escames G, López LC, Acuña-Castroviejo D. Díaz-Casado ME, et al. Zebrafish. 2018 Feb;15(1):15-26. doi: 10.1089/zeb.2017.1479. Epub 2017 Nov 29. Zebrafish. 2018. PMID: 29185873
Coenzyme Q10 modulates sulfide metabolism and links the mitochondrial respiratory chain to pathways associated to one carbon metabolism.
González-García P, Hidalgo-Gutiérrez A, Mascaraque C, Barriocanal-Casado E, Bakkali M, Ziosi M, Abdihankyzy UB, Sánchez-Hernández S, Escames G, Prokisch H, Martín F, Quinzii CM, López LC. González-García P, et al. Among authors: hidalgo gutierrez a. Hum Mol Genet. 2020 Nov 25;29(19):3296-3311. doi: 10.1093/hmg/ddaa214. Hum Mol Genet. 2020. PMID: 32975579 Free PMC article.
Metabolic Targets of Coenzyme Q10 in Mitochondria.
Hidalgo-Gutiérrez A, González-García P, Díaz-Casado ME, Barriocanal-Casado E, López-Herrador S, Quinzii CM, López LC. Hidalgo-Gutiérrez A, et al. Antioxidants (Basel). 2021 Mar 26;10(4):520. doi: 10.3390/antiox10040520. Antioxidants (Basel). 2021. PMID: 33810539 Free PMC article. Review.
Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings.
González-García P, Barriocanal-Casado E, Díaz-Casado ME, López-Herrador S, Hidalgo-Gutiérrez A, López LC. González-García P, et al. Among authors: hidalgo gutierrez a. Antioxidants (Basel). 2021 Oct 26;10(11):1687. doi: 10.3390/antiox10111687. Antioxidants (Basel). 2021. PMID: 34829558 Free PMC article. Review.
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