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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.
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.
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: barriocanal casado e. Hum Mol Genet. 2020 Nov 25;29(19):3296-3311. doi: 10.1093/hmg/ddaa214. Hum Mol Genet. 2020. PMID: 32975579 Free PMC article.
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: barriocanal casado e. Antioxidants (Basel). 2021 Oct 26;10(11):1687. doi: 10.3390/antiox10111687. Antioxidants (Basel). 2021. PMID: 34829558 Free PMC article. Review.
Isogenic GAA-KO Murine Muscle Cell Lines Mimicking Severe Pompe Mutations as Preclinical Models for the Screening of Potential Gene Therapy Strategies.
Aguilar-González A, González-Correa JE, Barriocanal-Casado E, Ramos-Hernández I, Lerma-Juárez MA, Greco S, Rodríguez-Sevilla JJ, Molina-Estévez FJ, Montalvo-Romeral V, Ronzitti G, Sánchez-Martín RM, Martín F, Muñoz P. Aguilar-González A, et al. Among authors: barriocanal casado e. Int J Mol Sci. 2022 Jun 4;23(11):6298. doi: 10.3390/ijms23116298. Int J Mol Sci. 2022. PMID: 35682977 Free PMC article.
Mitochondrial impairment and melatonin protection in parkinsonian mice do not depend of inducible or neuronal nitric oxide synthases.
López A, Ortiz F, Doerrier C, Venegas C, Fernández-Ortiz M, Aranda P, Díaz-Casado ME, Fernández-Gil B, Barriocanal-Casado E, Escames G, López LC, Acuña-Castroviejo D. López A, et al. PLoS One. 2017 Aug 11;12(8):e0183090. doi: 10.1371/journal.pone.0183090. eCollection 2017. PLoS One. 2017. PMID: 28800639 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. Among authors: barriocanal casado e. Antioxidants (Basel). 2021 Mar 26;10(4):520. doi: 10.3390/antiox10040520. Antioxidants (Basel). 2021. PMID: 33810539 Free PMC article. Review.
15 results