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Page 1
Genetic Rescue of Mitochondrial and Skeletal Muscle Impairment in an Induced Pluripotent Stem Cells Model of Coenzyme Q10 Deficiency.
Romero-Moya D, Santos-Ocaña C, Castaño J, Garrabou G, Rodríguez-Gómez JA, Ruiz-Bonilla V, Bueno C, González-Rodríguez P, Giorgetti A, Perdiguero E, Prieto C, Moren-Nuñez C, Fernández-Ayala DJ, Victoria Cascajo M, Velasco I, Canals JM, Montero R, Yubero D, Jou C, López-Barneo J, Cardellach F, Muñoz-Cánoves P, Artuch R, Navas P, Menendez P. Romero-Moya D, et al. Stem Cells. 2017 Jul;35(7):1687-1703. doi: 10.1002/stem.2634. Epub 2017 May 23. Stem Cells. 2017. PMID: 28472853 Free article.
The mitochondrial phosphatase PPTC7 orchestrates mitochondrial metabolism regulating coenzyme Q10 biosynthesis.
González-Mariscal I, Martin-Montalvo A, Vazquez-Fonseca L, Pomares-Viciana T, Sánchez-Cuesta A, Fernández-Ayala DJ, Navas P, Santos-Ocana C. González-Mariscal I, et al. Among authors: fernandez ayala dj. Biochim Biophys Acta Bioenerg. 2018 Nov;1859(11):1235-1248. doi: 10.1016/j.bbabio.2018.09.369. Epub 2018 Sep 26. Biochim Biophys Acta Bioenerg. 2018. PMID: 30267671 Free article.
Modelling the human Coenzyme Q deficiency in Drosophila melanogaster.
Fernández-Ayala DJM, Jiménez-Gancedo S, Guerra I, Hernández-Camacho JD, Neto M, Scialo F, Astillero-López V, Cortés-Rodríguez AB, Santos-Ocaña C, Rodríguez-Aguilera JC, Casares F, Sanz A, López-Lluch G, Navas P. Fernández-Ayala DJM, et al. Free Radic Biol Med. 2025 Jan 24:S0891-5849(24)01165-1. doi: 10.1016/j.freeradbiomed.2024.12.056. Online ahead of print. Free Radic Biol Med. 2025. PMID: 39864756
Identification of a new COQ4 spliceogenic variant causing severe primary coenzyme Q deficiency.
Alcázar-Fabra M, Østergaard E, Fernández-Ayala DJM, Desbats MA, Morbidoni V, Tomás-Gallado L, García-Corzo L, Blanquer-Roselló MDM, Bartlett AK, Sánchez-Cuesta A, Sena L, Cortés-Rodríguez A, Cascajo-Almenara MV, Pagliarini DJ, Trevisson E, Gronborg SW, Brea-Calvo G. Alcázar-Fabra M, et al. Among authors: fernandez ayala djm. Mol Genet Metab Rep. 2024 Dec 14;42:101176. doi: 10.1016/j.ymgmr.2024.101176. eCollection 2025 Mar. Mol Genet Metab Rep. 2024. PMID: 39759098 Free PMC article.
Prenatal and progressive coenzyme Q10 administration to mitigate muscle dysfunction in mitochondrial disease.
Hernández-Camacho JD, Vicente-García C, Ardila-García L, Padilla-Campos A, López-Lluch G, Santos-Ocaña C, Zammit PS, Carvajal JJ, Navas P, Fernández-Ayala DJM. Hernández-Camacho JD, et al. Among authors: fernandez ayala djm. J Cachexia Sarcopenia Muscle. 2024 Dec;15(6):2402-2416. doi: 10.1002/jcsm.13574. Epub 2024 Oct 2. J Cachexia Sarcopenia Muscle. 2024. PMID: 39354863 Free PMC article.
Calorie Restriction Rescues Mitochondrial Dysfunction in Adck2-Deficient Skeletal Muscle.
Hernández-Camacho JD, Fernández-Ayala DJM, Vicente-García C, Navas-Enamorado I, López-Lluch G, Oliva C, Artuch R, Garcia-Villoria J, Ribes A, de Cabo R, Carvajal JJ, Navas P. Hernández-Camacho JD, et al. Among authors: fernandez ayala djm. Front Physiol. 2022 Jul 14;13:898792. doi: 10.3389/fphys.2022.898792. eCollection 2022. Front Physiol. 2022. PMID: 35936917 Free PMC article.
33 results