Cell biology and Biotechnology

Gene expression and development in Coenzyme Q deficiency: a model to establish the origin of this mitochondrial disease

Dr Daniel José Moreno Fernández-Ayala
Researcher associated to Dr Carlos Santos Ocaña

Summary
Relevant publications
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Relevant publications

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. Calorie Restriction Rescues Mitochondrial Dysfunction in Adck2-Deficient Skeletal Muscle. Front Physiol. 2022 Jul 14;13:898792. doi: 10.3389/fphys.2022.898792. PMID: 35936917; PMCID: PMC9351392.

Hernández-Camacho JD, Vicente-García C, Sánchez-Cuesta A, Fernandez-Ayala DJM, Carvajal JJ, Navas P. Isolation of Mitochondria from Mouse Skeletal Muscle for Respirometric Assays. J Vis Exp. 2022 Feb 10;(180). doi: 10.3791/63336. PMID: 35225269.

Vázquez-Fonseca L, Schaefer J, Navas-Enamorado I, Santos-Ocaña C, Hernández-Camacho JD, Guerra I, Cascajo MV, Sánchez-Cuesta A, Horvath Z, Siendones E, Jou C, Casado M, Gutiérrez P, Brea-Calvo G, López-Lluch G, Fernández-Ayala DJM, Cortés-Rodríguez AB, Rodríguez-Aguilera JC, Matté C, Ribes A, Prieto-Soler SY, Dominguez-Del-Toro E, Francesco AD, Aon MA, Bernier M, Salviati L, Artuch R, Cabo R, Jackson S, Navas P. ADCK2 Haploinsufficiency Reduces Mitochondrial Lipid Oxidation and Causes Myopathy Associated with CoQ Deficiency. J Clin Med. 2019 Sep 2;8(9):1374. doi: 10.3390/jcm8091374. PMID: 31480808; PMCID: PMC6780728.

Rodríguez-Aguilera JC, Cortés AB, Fernández-Ayala DJ, Navas P. Biochemical Assessment of Coenzyme Q10 Deficiency. J Clin Med. 2017 Mar 5;6(3):27. doi: 10.3390/jcm6030027. PMID: 28273876; PMCID: PMC5372996.

Scialò F, Sriram A, Fernández-Ayala D, Gubina N, Lõhmus M, Nelson G, Logan A, Cooper HM, Navas P, Enríquez JA, Murphy MP, Sanz A. Mitochondrial ROS Produced via Reverse Electron Transport Extend Animal Lifespan. Cell Metab. 2016 Apr 12;23(4):725-34. doi: 10.1016/j.cmet.2016.03.009. PMID: 27076081; PMCID: PMC4835580.

Fernández-Ayala DJ, Guerra I, Jiménez-Gancedo S, Cascajo MV, Gavilán A, Dimauro S, Hirano M, Briones P, Artuch R, De Cabo R, Salviati L, Navas P. Survival transcriptome in the coenzyme Q10 deficiency syndrome is acquired by epigenetic modifications: a modeling study for human coenzyme Q10 deficiencies. BMJ Open. 2013 Mar 25;3(3):e002524. doi: 10.1136/bmjopen-2012-002524. PMID: 23533218; PMCID: PMC3612821.

Fernández-Ayala DJ, Chen S, Kemppainen E, O'Dell KM, Jacobs HT. Gene expression in a Drosophila model of mitochondrial disease. PLoS One. 2010 Jan 6;5(1):e8549. doi: 10.1371/journal.pone.0008549. PMID: 20066047; PMCID: PMC2798955.

Fernandez-Ayala DJ, Sanz A, Vartiainen S, Kemppainen KK, Babusiak M, Mustalahti E, Costa R, Tuomela T, Zeviani M, Chung J, O'Dell KM, Rustin P, Jacobs HT. Expression of the Ciona intestinalis alternative oxidase (AOX) in Drosophila complements defects in mitochondrial oxidative phosphorylation. Cell Metab. 2009 May;9(5):449-60. doi: 10.1016/j.cmet.2009.03.004. PMID: 19416715.

Fernández-Ayala DJ, Brea-Calvo G, López-Lluch G, Navas P. Coenzyme Q distribution in HL-60 human cells depends on the endomembrane system. Biochim Biophys Acta. 2005 Jul 30;1713(2):129-37. doi: 10.1016/j.bbamem.2005.05.010. PMID: 15993380.

Fernández-Ayala DJ, López-Lluch G, García-Valdés M, Arroyo A, Navas P. Specificity of coenzyme Q10 for a balanced function of the respiratory chain and endogenous ubiquinone biosynthesis in human cells. Biochim Biophys Acta. 2005 Jan 7;1706(1-2):174-83. doi: 10.1016/j.bbabio.2004.10.009. PMID: 15620378.

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