C orral-Sarasa J, Martínez-Gálvez JM, González-García P, Wendling O, Jiménez-Sánchez L, López-Herrador S, Quinzii CM, Díaz-Casado ME, López LC.
4-Hydroxybenzoic acid rescues multisystemic disease and perinatal lethality in a mouse model of mitochondrial disease. Cell Rep. 2024 Apr 26:114148. doi: 10.1016/j.celrep.2024.114148.

A ragón-Vela J, Casuso RA, Aparisi AS, Plaza-Díaz J, Rueda-Robles A, Hidalgo-Gutiérrez A, López LC, Rodríguez-Carrillo A, Enriquez JA, Cogliati S, Huertas JR.

Early heart and skeletal muscle mitochondrial response to a moderate hypobaric hypoxia environment. J Physiol. 2024 Apr 17. doi: 10.1113/JP285516.

M onfrini E, Pesini A, Biella F, Sobreira CFR, Emmanuele V, Brescia G, Lopez LC, Tadesse S, Hirano M, Comi GP, Quinzii CM, Di Fonzo A.
Whole-Exome Sequencing Study of Fibroblasts Derived From Patients With Cerebellar Ataxia Referred to Investigate CoQ10 Deficiency. Neurol Genet. 2023 Mar 14;9(2):e200058. doi: 10.1212/NXG.0000000000200058.


Florido J, Martinez-Ruiz L, Rodriguez-Santana C, López-Rodríguez A, Hidalgo-Gutiérrez A, Cottet-Rousselle C, Lamarche F, Schlattner U, Guerra-Librero A, Aranda-Martínez P, Acuña-Castroviejo D, López LC, Escames G.
Melatonin drives apoptosis in head and neck cancer by increasing mitochondrial ROS generated via reverse electron transport. J Pineal Res. 2022 Oct;73(3):e12824. doi: 10.1111/jpi.12824.

González-García P, Díaz-Casado ME, Hidalgo-Gutiérrez A, Jiménez-Sánchez L, Bakkali M, Barriocanal-Casado E, Escames G, Chiozzi RZ, Völlmy F, Zaal EA, Berkers CR, Heck AJR, López LC.
The Q-junction and the inflammatory response are critical pathological and therapeutic factors in CoQ deficiency. Redox Biol. 2022 Jul 15;55:102403. doi: 10.1016/j.redox.2022.102403.

Rodríguez-Carrillo A, D’Cruz SC, Mustieles V, Suárez B, Smagulova F, David A, Peinado F, Artacho-Cordón F, López LC, Arrebola JP, Olea N, Fernández MF, Freire C.
Exposure to non-persistent pesticides, BDNF, and behavioral function in adolescent males: Exploring a novel effect biomarker approach. Environ Res. 2022 Aug;211:113115. doi: 10.1016/j.envres.2022.113115.


González-García P, Barriocanal-Casado E, Díaz-Casado ME, López-Herrador S, Hidalgo-Gutiérrez A, López LC.
Animal Models of Coenzyme Q Deficiency: Mechanistic and Translational Learnings. Antioxidants (Basel). 2021 Oct 26;10(11):1687. doi: 10.3390/antiox10111687.

Hidalgo-Gutiérrez A, Barriocanal-Casado E, Díaz-Casado ME, González-García P, Zenezini Chiozzi R, Acuña-Castroviejo D, López LC.
β-RA Targets Mitochondrial Metabolism and Adipogenesis, Leading to Therapeutic Benefits against CoQ Deficiency and Age-Related Overweight. Biomedicines. 2021 Oct 13;9(10):1457. doi: 10.3390/biomedicines9101457.

Hidalgo-Gutiérrez A, González-García P, Díaz-Casado ME, Barriocanal-Casado E, López-Herrador S, Quinzii CM, López LC.
Metabolic Targets of Coenzyme Q10 in Mitochondria. Antioxidants (Basel). 2021 Mar 26;10(4):520. doi: 10.3390/antiox10040520.

Quinzii CM, Lopez LC.
Abnormalities of hydrogen sulfide and glutathione pathways in mitochondrial dysfunction. J Adv Res. 2020 Apr 7;27:79-84. doi: 10.1016/j.jare.2020.04.002.

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.
Coenzyme Q10 modulates sulfide metabolism and links the mitochondrial respiratory chain to pathways associated to one carbon metabolism. Hum Mol Genet. 2020 Nov 25;29(19):3296-3311. doi: 10.1093/hmg/ddaa214.

Barriocanal-Casado E, Hidalgo-Gutiérrez A, Raimundo N, González-García P, Acuña-Castroviejo D, Escames G, López LC.
Rapamycin administration is not a valid therapeutic strategy for every case of mitochondrial disease. EBioMedicine. 2019 Mar 18. pii: S2352-3964(19)30165-3. doi: 10.1016/j.ebiom.2019.03.025.

Fernandez-Gil BI, Guerra-Librero A, Shen YQ, Florido J, Martínez-Ruiz L, García-López S, Adan C, Rodríguez-Santana C, Acuña-Castroviejo D, Quiñones-Hinojosa A, Fernández-Martínez J, Abdel Moneim AE, López LC, Rodríguez Ferrer JM, Escames G.
Melatonin Enhances Cisplatin and Radiation Cytotoxicity in Head and Neck Squamous Cell Carcinoma by Stimulating Mitochondrial ROS Generation, Apoptosis, and Autophagy. Oxid Med Cell Longev. 2019 Feb 28;2019:7187128. doi: 10.1155/2019/7187128.

Sayed RKA, Fernández-Ortiz M, Diaz-Casado ME, Aranda-Martínez P, Fernández-Martínez J, Guerra-Librero A, Escames G, López LC, Alsaadawy RM, Acuña-Castroviejo D.
Lack of NLRP3 inflammasome activation reduces age-dependent sarcopenia and mitochondrial dysfunction, favoring the prophylactic effect of melatonin. EJ Gerontol A Biol Sci Med Sci. 2019 Mar 14. pii: glz079. doi: 10.1093/gerona/glz079.

Casuso RA, Al-Fazazi S, Hidalgo-Gutierrez A, López LC, Plaza-Díaz J, Rueda-Robles A, Huertas JR.
Hydroxytyrosol influences exercise-induced mitochondrial respiratory complex assembly into supercomplexes in rats. Free Radic Biol Med. 2019 Jan 24;134:304-310. doi: 10.1016/j.freeradbiomed.2019.01.027.

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.
β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9 R239X mice. EMBO Mol Med. 2019 Jan;11(1). pii: e9466. doi: 10.15252/emmm.201809466.


Kleiner G, Barca E, Ziosi M, Emmanuele V, Xu Y, Hidalgo-Gutierrez A, Qiao C, Tadesse S, Area-Gomez E, Lopez LC, Quinzii CM.
CoQ10 supplementation rescues nephrotic syndrome through normalization of H2S oxidation pathway. Biochim Biophys Acta Mol Basis Dis. 2018 Nov;1864(11):3708-3722. doi: 10.1016/j.bbadis.2018.09.002.

Rodríguez-Hidalgo M, Luna-Sánchez M, Hidalgo-Gutiérrez A, Barriocanal-Casado E, Mascaraque C, Acuña-Castroviejo D, Rivera M, Escames G, López LC.
Reduction in the levels of CoQ biosynthetic proteins is related to an increase in lifespan without evidence of hepatic mitohormesis. Sci Rep. 2018 Sep 18;8(1):14013. doi: 10.1038/s41598-018-32190-y.

Sayed RKA, Fernández-Ortiz M, Diaz-Casado ME, Rusanova I, Rahim I, Escames G, López LC, Mokhtar DM, Acuña-Castroviejo D.
The Protective Effect of Melatonin Against Age-Associated, Sarcopenia-Dependent Tubular Aggregate Formation, Lactate Depletion, and Mitochondrial Changes. J Gerontol A Biol Sci Med Sci. 2018 Sep 11;73(10):1330-1338. doi: 10.1093/gerona/gly059.

Shen YQ, Guerra-Librero A, Fernandez-Gil BI, Florido J, García-López S, Martinez-Ruiz L, Mendivil-Perez M, Soto-Mercado V, Acuña-Castroviejo D, Ortega-Arellano H, Carriel V, Diaz-Casado ME, Reiter RJ, Rusanova I, Nieto A, López LC, Escames G.
Combination of melatonin and rapamycin for head and neck cancer therapy: Suppression of AKT/mTOR pathway activation, and activation of mitophagy and apoptosis via mitochondrial function regulation. J Pineal Res. 2018 Apr;64(3). doi: 10.1111/jpi.12461.

Herebian D, López LC, Distelmaier F.
Bypassing human CoQ10 deficiency. Mol Genet Metab. 2018 Mar;123(3):289-291. doi: 10.1016/j.ymgme.2017.12.008. Epub 2017

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.
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. Zebrafish. 2018 Feb;15(1):15-26. doi: 10.1089/zeb.2017.1479.


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.
Mitochondrial impairment and melatonin protection in parkinsonian mice do not depend of inducible or neuronal nitric oxide synthases. PLoS One. 2017 Aug 11;12(8):e0183090.

Quinzii CM, Luna-Sanchez M, Ziosi M, Hidalgo-Gutierrez A, Kleiner G, Lopez LC.
The Role of Sulfide Oxidation Impairment in the Pathogenesis of Primary CoQ Deficiency. Front Physiol. 2017 Jul 25;8:525.

Acuña-Castroviejo D, Rahim I, Acuña-Fernández C, Fernández-Ortiz M, Solera-Marín J, Sayed RKA, Díaz-Casado ME, Rusanova I, López LC, Escames G.
Melatonin, clock genes and mitochondria in sepsis. Cell Mol Life Sci. 2017 Nov;74(21):3965-3987.

Huertas JR, Al Fazazi S, Hidalgo-Gutierrez A, López LC, Casuso RA.
Antioxidant effect of exercise: Exploring the role of the mitochondrial complex I superassembly. Redox Biol. 2017 Oct;13:477-481

Herebian D, Seibt A, Smits SHJ, Bünning G, Freyer C, Prokisch H, Karall D, Wredenberg A, Wedell A, López LC, Mayatepek E, Distelmaier F.
Detection of 6-demethoxyubiquinone in CoQ10 deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics. Mol Genet Metab. 2017 Jul;121(3):216-223.

Mendivil-Perez M, Soto-Mercado V, Guerra-Librero A, Fernandez-Gil BI, Florido J, Shen YQ, Tejada MA, Capilla-Gonzalez V, Rusanova I, Garcia-Verdugo JM, Acuña-Castroviejo D, López LC, Velez-Pardo C, Jimenez-Del-Rio M, Ferrer JM, Escames G.
Melatonin enhances neural stem cell differentiation and engraftment by increasing mitochondrial function. J Pineal Res. 2017 Sep;63(2). doi: 10.1111/jpi.12415.

Fernández-Gil B, Moneim AE, Ortiz F, Shen YQ, Soto-Mercado V, Mendivil-Perez M, Guerra-Librero A, Acuña-Castroviejo D, Molina-Navarro MM, García-Verdugo JM, Sayed RK, Florido J, Luna JD, López LC, Escames G.
Melatonin protects rats from radiotherapy-induced small intestine toxicity. PLoS One. 2017 Apr 12;12(4):e0174474.

Rahim I, Djerdjouri B, Sayed RK, Fernández-Ortiz M, Fernández-Gil B, Hidalgo-Gutiérrez A, López LC, Escames G, Reiter RJ, Acuña-Castroviejo D.
Melatonin administration to wild-type mice and nontreated NLRP3 mutant mice share similar inhibition of the inflammatory response during sepsis. J Pineal Res. 2017 Aug;63(1). doi: 10.1111/jpi.12410.

Fernández-Mosquera L, Diogo CV, Yambire KF, Santos GL, Luna Sánchez M, Bénit P, Rustin P, Lopez LC, Milosevic I, Raimundo N.
Acute and chronic mitochondrial respiratory chain deficiency differentially regulate lysosomal biogenesis. Sci Rep. 2017 Mar 27;7:45076. doi: 10.1038/srep45076.

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.
CoQ deficiency causes disruption of mitochondrial sulfide oxidation, a new pathomechanism associated with this syndrome. EMBO Mol Med. 2017 Nov 17; 9(1):78-95. pii: e201606345. doi: 10.15252/emmm.201606345.


Barriocanal-Casado E, Cueto-Ureña C, Benabdellah K, Gutiérrez-Guerrero A, Cobo M, Hidalgo-Gutiérrez A, Rodríguez-Sevilla JJ, Martín F, López LC.
Gene Therapy Corrects Mitochondrial Dysfunction in Hematopoietic Progenitor Cells and Fibroblasts from Coq9R239X Mice. PLoS One. 2016 Jun 24;11(6):e0158344. doi: 10.1371/journal.pone.0158344.

Volt H, García JA, Doerrier C, Díaz-Casado ME, Guerra-Librero A, López LC, Escames G, Tresguerres JA, Acuña-Castroviejo D.
Same molecule but different expression: aging and sepsis trigger NLRP3 inflammasome activation, a target of melatonin. J Pineal Res. 2015 Dec 17. doi: 10.1111/jpi.12303.

García JA, Volt H, Venegas C, Doerrier C, Escames G, López LC, Acuña-Castroviejo D.
Disruption of the NF-κB/NLRP3 connection by melatonin requires retinoid-related orphan receptor-α and blocks the septic response in mice. FASEB J. 2015 Sep;29(9):3863-75. doi: 10.1096/fj.15-273656.

Garcia-Diaz B, Barca E, Balreira A, Lopez LC, Tadesse S, Krishna S, Naini A, Mariotti C, Castellotti B, Quinzii CM.
Lack of aprataxin impairs mitochondrial functions via down-regulation of the APE1/ NRF1/NRF2 pathway. Hum Mol Genet. 2015 Aug 15;24(16):4516-29. doi: 10.1093/hmg/ddv183.

Luna‐Sánchez M, Díaz‐Casado E, Barca E, Tejada MA, Montilla‐García A, Cobos EJ, Escames G, Acuña‐Castroviejo D, Quinzii CM, López LC.
The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene. EMBO Mol Med. 2015 Mar 23;7(5):670-87. doi: 10.15252/emmm.201404632.

Ortiz F, Acuña-Castroviejo D, Doerrier C, Dayoub JC, López LC, Venegas C, García JA, López A, Volt H, Luna-Sánchez M, Escames G.
Melatonin blunts the mitochondrial/NLRP3 connection and protects against radiation-induced oral mucositis. J Pineal Res. 2015 Jan;58(1):34-49. doi: 10.1111/jpi.12191. Epub 2014 Dec 4. PMID: 25388914


Lohman DC, Forouhar F, Beebe ET, Stefely MS, Minogue CE, Ulbrich A, Stefely JA, Sukumar S, Luna-Sánchez M, Jochem A, Lew S, Seetharaman J, Xiao R, Wang H, Westphall MS, Wrobel RL, Everett JK, Mitchell JC, López LC, Coon JJ, Tong L, Pagliarini DJ.
COQ9 is a lipid-binding protein that associates with COQ7 to enable coenzyme Q biosynthesis. Proc Natl Acad Sci U S A. 2014 Oct 22. pii: 201413128. [Epub ahead of print] PMID: 25339443

López LC, Luna-Sánchez M, García-Corzo L, Quinzii CM, Hirano M.
Pathomechanisms in coenzyme q10-deficient human fibroblasts. Mol Syndromol. 2014 Jul;5(3-4):163-9. doi: 10.1159/000360494. PubMed PMID: 25126049; PubMed Central PMCID: PMC4112524.

Garone C, Garcia-Diaz B, Emmanuele V, Lopez LC, Tadesse S, Akman HO, Tanji K, Quinzii CM, Hirano M.
Deoxypyrimidine monophosphate bypass therapy for thymidine kinase 2 deficiency. EMBO Mol Med. 2014 Jun 26;6(8):1016-27. doi:10.15252/emmm.201404092. PubMed PMID: 24968719; PubMed Central PMCID: PMC4154130.

Lima-Cabello E, Díaz-Casado ME, Guerrero JA, Otalora BB, Escames G, López LC, Reiter RJ, Acuña-Castroviejo D.
A review of the melatonin functions in zebrafish physiology. J Pineal Res. 2014 Aug;57(1):1-9. doi: 10.1111/jpi.12149. Epub 2014Jul 3. PubMed PMID: 24920150.

García-Corzo L, Luna-Sánchez M, Doerrier C, Ortiz F, Escames G, Acuña-Castroviejo D, López LC.
Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency. Biochim Biophys Acta. 2014 Feb24;1842(7):893-901. doi: 10.1016/j.bbadis.2014.02.008. [Epub ahead of print]PubMed PMID: 24576561.

Acuña-Castroviejo D, Escames G, Venegas C, Díaz-Casado ME, Lima-Cabello E, López LC, Rosales-Corral S, Tan DX, Reiter RJ.
Extrapineal melatonin: sources,regulation, and potential functions. Cell Mol Life Sci. 2014 Feb 20. [Epub ahead of print] PubMed PMID: 24554058.

Ortiz F, García JA, Acuña-Castroviejo D, Doerrier C, López A, Venegas C, Volt H, Luna-Sánchez M, López LC, Escames G.
The beneficial effects of melatonin against heart mitochondrial impairment during sepsis: inhibition of iNOS and preservation of nNOS. J Pineal Res. 2014 Jan;56(1):71-81. doi: 10.1111/jpi.12099. Epub 2013 Oct 21. PubMed PMID: 24117944.

López LC, Cabrera-Vique C, Venegas C, García-Corzo L, Luna-Sánchez M, Acuña-Castroviejo D, Escames G.
Argan oil-contained antioxidants for human mitochondria. Nat Prod Commun. 2013 Jan;8(1):47-50. Review. PubMed PMID: 23472457.

Romero-Moya D, Bueno C, Montes R, Navarro-Montero O, Iborra FJ, López LC, Martin M, Menendez P.
Cord blood-derived CD34+ hematopoietic cells with low mitochondrial mass are enriched in hematopoietic repopulating stem cell function. Haematologica. 2013 Jul;98(7):1022-9. doi: 10.3324/haematol.2012.079244. Epub 2013 Jan 24. PubMed PMID: 23349299; PubMed Central PMCID: PMC3696604.

García-Corzo L, Luna-Sánchez M, Doerrier C, García JA, Guarás A, Acín-Pérez R, Bullejos-Peregrín J, López A, Escames G, Enríquez JA, Acuña-Castroviejo D, López LC.
Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency. Hum Mol Genet. 2013 Mar 15;22(6):1233-48. doi:10.1093/hmg/dds530. Epub 2012 Dec 18. PubMed PMID: 23255162.

Venegas C, García JA, Doerrier C, Volt H, Escames G, López LC, Reiter RJ, Acuña-Castroviejo D.
Analysis of the daily changes of melatonin receptors in the rat liver. J Pineal Res. 2013 Apr;54(3):313-21. doi: 10.1111/jpi.12019. Epub 2012 Oct 30. PubMed PMID: 23110416.


Emmanuele V, López LC, Berardo A, Naini A, Tadesse S, Wen B, D’Agostino E, Solomon M, DiMauro S, Quinzii C, Hirano M.
Heterogeneity of coenzyme Q10 deficiency: patient study and literature review. Arch Neurol. 2012 Aug;69(8):978-83. doi: 10.1001/archneurol.2012.206. Review. Erratum in: Arch Neurol. 2012 Jul;69(7):886. López, Luis [corrected to López, Luis C]. PubMedPMID: 22490322; PubMed Central PMCID: PMC3639472.

Martí R, López LC, Hirano M.
Assessment of thymidine phosphorylase function: measurement of plasma thymidine (and deoxyuridine) and thymidine phosphorylase activity. Methods Mol Biol. 2012;837:121-33. doi: 10.1007/978-1-61779-504-6_8. PubMed PMID: 22215544.

Venegas C, Cabrera-Vique C, García-Corzo L, Escames G, Acuña-Castroviejo D, López LC.
Determination of coenzyme Q10, coenzyme Q9, and melatonin contents in virgin argan oils: comparison with other edible vegetable oils. J Agric Food Chem. 2011 Nov 23;59(22):12102-8. doi: 10.1021/jf203428t. Epub 2011 Oct 31. PubMed PMID: 22007968.

Venegas C, García JA, Escames G, Ortiz F, López A, Doerrier C, García-Corzo L, López LC, Reiter RJ, Acuña-Castroviejo D.
Extrapineal melatonin: analysis of its subcellular distribution and daily fluctuations. J Pineal Res. 2012 Mar;52(2):217-27. doi: 10.1111/j.1600-079X.2011.00931.x. Epub 2011 Sep 2. PubMed PMID: 21884551.

Escames G, López LC, García JA, García-Corzo L, Ortiz F, Acuña-Castroviejo D.
Mitochondrial DNA and inflammatory diseases. Hum Genet. 2012 Feb;131(2):161-73. doi: 10.1007/s00439-011-1057-y. Epub 2011 Jul 7. Review. PubMed PMID: 21735170.

Acuña-Castroviejo D, Carretero M, Doerrier C, López LC, García-Corzo L, Tresguerres JA, Escames G.
Melatonin protects lung mitochondria from aging. Age (Dordr). 2012 Jun;34(3):681-92. doi: 10.1007/s11357-011-9267-8. Epub 2011 May 26. PubMed PMID: 21614449; PubMed Central PMCID: PMC3337938.

Dayoub JC, Ortiz F, López LC, Venegas C, Del Pino-Zumaquero A, Roda O, Sánchez-Montesinos I, Acuña-Castroviejo D, Escames G.
Synergism between melatonin and atorvastatin against endothelial cell damage induced by lipopolysaccharide. J Pineal Res. 2011 Oct;51(3):324-30. doi: 10.1111/j.1600-079X.2011.00892.x. Epub 2011 May 17. PubMed PMID: 21585521.

Chahbouni M, Escames G, López LC, Sevilla B, Doerrier C, Muñoz-Hoyos A, Molina-Carballo A, Acuña-Castroviejo D.
Melatonin treatment counteracts the hyperoxidative status in erythrocytes of patients suffering from Duchenne muscular dystrophy. Clin Biochem. 2011 Jul;44(10-11):853-8. doi: 10.1016/j.clinbiochem.2011.04.001. Epub 2011 Apr 15. PubMed PMID: 21515247.

Acuña-Castroviejo D, Tapias V, López LC, Doerrier C, Camacho E, Carrión MD, Mora F, Espinosa A, Escames G.
Protective effects of synthetic kynurenines on 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-induced parkinsonism in mice. Brain Res Bull. 2011 May 30;85(3-4):133-40. doi: 10.1016/j.brainresbull.2011.03.008. Epub 2011 Mar 17. PubMed PMID: 21419832.

Escames G, López A, García JA, García L, Acuña-Castroviejo D, García JJ, López LC.
The role of mitochondria in brain aging and the effects of melatonin. Curr Neuropharmacol. 2010 Sep;8(3):182-93. doi: 10.2174/157015910792246245. PubMed PMID: 21358969; PubMed Central PMCID: PMC3001212.

Acuña Castroviejo D, López LC, Escames G, López A, García JA, Reiter RJ.
Melatonin-mitochondria interplay in health and disease. Curr Top Med Chem. 2011;11(2):221-40. Review. PubMed PMID: 21244359.

Castroviejo DA, López LC, Escames G, López A, García JA, Reiter RJ.
Melatonin-mitochondria Interplay in Health and Disease. Curr Top Med Chem. 2010 Dec 28. [Epub ahead of print] PubMed PMID: 21192780.


López LC, Quinzii CM, Area E, Naini A, Rahman S, Schuelke M, Salviati L, Dimauro S, Hirano M.
Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects. PLoS One. 2010 Jul 30;5(7):e11897. doi: 10.1371/journal.pone.0011897. PubMed PMID: 20689595; PubMed Central PMCID: PMC2912846.

Quinzii CM, López LC, Gilkerson RW, Dorado B, Coku J, Naini AB, Lagier-Tourenne C, Schuelke M, Salviati L, Carrozzo R, Santorelli F, Rahman S, Tazir M, Koenig M, DiMauro S, Hirano M.
Reactive oxygen species, oxidative stress, and cell death correlate with level of CoQ10 deficiency. FASEB J. 2010 Oct;24(10):3733-43. doi: 10.1096/fj.09-152728. Epub 2010 May 21. PubMed PMID: 20495179; PubMed Central PMCID: PMC2996902.

Chahbouni M, Escames G, Venegas C, Sevilla B, García JA, López LC, Muñoz-Hoyos A, Molina-Carballo A, Acuña-Castroviejo D.
Melatonin treatment normalizes plasma pro-inflammatory cytokines and nitrosative/oxidative stress in patients suffering from Duchenne muscular dystrophy. J Pineal Res. 2010 Apr;48(3):282-9. doi: 10.1111/j.1600-079X.2010.00752.x. Epub 2010 Mar 4. PubMed PMID: 20210854.

Carretero M, Escames G, López LC, Venegas C, Dayoub JC, García L, Acuña-Castroviejo D.
Long-term melatonin administration protects brain mitochondria from aging. J Pineal Res. 2009 Sep;47(2):192-200. doi: 10.1111/j.1600-079X.2009.00700.x. Epub 2009 Jul 1. PubMed PMID: 19573039.

Tapias V, Escames G, López LC, López A, Camacho E, Carrión MD, Entrena A, Gallo MA, Espinosa A, Acuña-Castroviejo D.
Melatonin and its brain metabolite N(1)-acetyl-5-methoxykynuramine prevent mitochondrial nitric oxide synthase induction in parkinsonian mice. J Neurosci Res. 2009 Oct;87(13):3002-10. doi:10.1002/jnr.22123. PubMed PMID: 19437546.

Duncan AJ, Bitner-Glindzicz M, Meunier B, Costello H, Hargreaves IP, López LC, Hirano M, Quinzii CM, Sadowski MI, Hardy J, Singleton A, Clayton PT, Rahman S.
A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: a potentially treatable form of mitochondrial disease.Am J Hum Genet. 2009 May;84(5):558-66. doi: 10.1016/j.ajhg.2009.03.018. Epub 2009 Apr 16. PubMed PMID: 19375058; PubMed Central PMCID: PMC2681001.

Liang WC, Ohkuma A, Hayashi YK, López LC, Hirano M, Nonaka I, Noguchi S, Chen LH, Jong YJ, Nishino I.
ETFDH mutations, CoQ10 levels, and respiratory chain activities in patients with riboflavin-responsive multiple acyl-CoA dehydrogenase deficiency. Neuromuscul Disord. 2009 Mar;19(3):212-6. doi: 10.1016/j.nmd.2009.01.008. Epub 2009 Feb 26. PubMed PMID: 19249206.

Ohkuma A, Noguchi S, Sugie H, Malicdan MC, Fukuda T, Shimazu K, López LC, Hirano M, Hayashi YK, Nonaka I, Nishino I.
Clinical and genetic analysis of lipid storage myopathies. Muscle Nerve. 2009 Mar;39(3):333-42. doi: 10.1002/mus.21167. PubMed PMID: 19208393.

Quinzii CM, López LC, Naini A, DiMauro S, Hirano M.
Human CoQ10 deficiencies. Biofactors. 2008;32(1-4):113-8. PubMed PMID: 19096106; PubMed Central PMCID: PMC3625975.

López A, García JA, Escames G, Venegas C, Ortiz F, López LC, Acuña-Castroviejo D.
Melatonin protects the mitochondria from oxidative damage reducing oxygen consumption, membrane potential, and superoxide anion production.J Pineal Res. 2009 Mar;46(2):188-98. doi: 10.1111/j.1600-079X.2008.00647.x. Epub 2008 Nov 19. PubMed PMID: 19054298.

López LC, Akman HO, García-Cazorla A, Dorado B, Martí R, Nishino I, Tadesse S, Pizzorno G, Shungu D, Bonilla E, Tanji K, Hirano M.
Unbalanced deoxynucleotide pools cause mitochondrial DNA instability in thymidine phosphorylase-deficient mice. Hum Mol Genet. 2009 Feb 15;18(4):714-22. doi: 10.1093/hmg/ddn401. Epub 2008 Nov 21. PubMed PMID: 19028666; PubMed Central PMCID: PMC2638828.

Rodríguez MI, Escames G, López LC, López A, García JA, Ortiz F, Sánchez V, Romeu M, Acuña-Castroviejo D.
Improved mitochondrial function and increased life span after chronic melatonin treatment in senescent prone mice. Exp Gerontol. 2008 Aug;43(8):749-56. doi: 10.1016/j.exger.2008.04.003. Epub 2008 Apr 6. PubMed PMID: 18485648.

Akman HO, Dorado B, López LC, García-Cazorla A, Vilà MR, Tanabe LM, Dauer WT, Bonilla E, Tanji K, Hirano M.
Thymidine kinase 2 (H126N) knockin mice show the essential role of balanced deoxynucleotide pools for mitochondrial DNA maintenance. Hum Mol Genet. 2008 Aug 15;17(16):2433-40. doi: 10.1093/hmg/ddn143. Epub 2008 May 8. PubMed PMID: 18467430; PubMed Central PMCID: PMC3115590.

Lagier-Tourenne C, Tazir M, López LC, Quinzii CM, Assoum M, Drouot N, Busso C, Makri S, Ali-Pacha L, Benhassine T, Anheim M, Lynch DR, Thibault C, Plewniak F, Bianchetti L, Tranchant C, Poch O, DiMauro S, Mandel JL, Barros MH, Hirano M, Koenig M.
ADCK3, an ancestral kinase, is mutated in a form of recessive ataxia associated with coenzyme Q10 deficiency. Am J Hum Genet. 2008 Mar;82(3):661-72. doi: 10.1016/j.ajhg.2007.12.024. PubMed PMID: 18319074; PubMed Central PMCID: PMC2427193.

Quinzii CM, López LC, Von-Moltke J, Naini A, Krishna S, Schuelke M, Salviati L, Navas P, DiMauro S, Hirano M.
Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency. FASEB J. 2008 Jun;22(6):1874-85. doi: 10.1096/fj.07-100149. Epub 2008 Jan 29. PubMed PMID: 18230681; PubMed Central PMCID: PMC2731482.


Rodriguez MI, Escames G, López LC, García JA, Ortiz F, López A, Acuña-Castroviejo D.
Melatonin administration prevents cardiac and diaphragmatic mitochondrial oxidative damage in senescence-accelerated mice. J Endocrinol. 2007 Sep;194(3):637-43. PubMed PMID: 17761903.

García-Navarro A, González-Puga C, Escames G, López LC, López A, López-Cantarero M, Camacho E, Espinosa A, Gallo MA, Acuña-Castroviejo D.
Cellular mechanisms involved in the melatonin inhibition of HT-29 human colon cancer cell proliferation in culture. J Pineal Res. 2007 Sep;43(2):195-205. PubMed PMID:17645698.

Valentino ML, Martí R, Tadesse S, López LC, Manes JL, Lyzak J, Hahn A, Carelli V, Hirano M.
Thymidine and deoxyuridine accumulate in tissues of patients with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE). FEBS Lett. 2007 Jul 24;581(18):3410-4. Epub 2007 Jun 27. PubMed PMID: 17612528; PubMed Central PMCID: PMC1986782.

Escames G, López LC, Ortiz F, López A, García JA, Ros E, Acuña-Castroviejo D.
Attenuation of cardiac mitochondrial dysfunction by melatonin in septic mice. FEBS J. 2007 Apr;274(8):2135-47. Epub 2007 Mar 20. PubMed PMID: 17371545.

Rodríguez MI, Escames G, López LC, López A, García JA, Ortiz F, Acuña-Castroviejo D.
Chronic melatonin treatment reduces the age-dependent inflammatory process in senescence-accelerated mice. J Pineal Res. 2007 Apr;42(3):272-9. PubMed PMID: 17349026.

López LC, Schuelke M, Quinzii CM, Kanki T, Rodenburg RJ, Naini A, Dimauro S, Hirano M.
Leigh syndrome with nephropathy and CoQ10 deficiency due to decaprenyl diphosphate synthase subunit 2 (PDSS2) mutations. Am J Hum Genet. 2006 Dec;79(6):1125-9. Epub 2006 Oct 27. PubMed PMID: 17186472; PubMed Central PMCID: PMC1698707.

Rodríguez MI, Carretero M, Escames G, López LC, Maldonado MD, Tan DX, Reiter RJ, Acuña-Castroviejo D.
Chronic melatonin treatment prevents age-dependent cardiac mitochondrial dysfunction in senescence-accelerated mice. Free Radic Res. 2007 Jan;41(1):15-24. PubMed PMID: 17164175.

López LC, Escames G, Ortiz F, Ros E, Acuña-Castroviejo D.
Melatonin restores the mitochondrial production of ATP in septic mice. Neuro Endocrinol Lett. 2006 Oct;27(5):623-30. PubMed PMID: 17159820.

Acuna-Castroviejo D, Escames G, Rodriguez MI, Lopez LC.
Melatonin role in the mitochondrial function. Front Biosci. 2007 Jan 1;12:947-63. Review. PubMed PMID: 17127351.

Escames G, López LC, Ortiz F, Ros E, Acuña-Castroviejo D.
Age-dependent lipopolysaccharide-induced iNOS expression and multiorgan failure in rats: effects of melatonin treatment. Exp Gerontol. 2006 Nov;41(11):1165-73. Epub 2006 Oct 17. PubMed PMID: 17049787.

Escames G, Acuña-Castroviejo D, López LC, Tan DX, Maldonado MD, Sánchez-Hidalgo M, León J, Reiter RJ.
Pharmacological utility of melatonin in the treatment of septic shock: experimental and clinical evidence. J Pharm Pharmacol. 2006 Sep;58(9):1153-65. Review. PubMed PMID: 16945173.

León J, Escames G, Rodríguez MI, López LC, Tapias V, Entrena A, Camacho E, Carrión MD, Gallo MA, Espinosa A, Tan DX, Reiter RJ, Acuña-Castroviejo D. Inhibition of neuronal nitric oxide synthase activity by N1-acetyl-5-methoxykynuramine, a brain metabolite of melatonin. J Neurochem. 2006 Sep;98(6):2023-33. PubMed PMID: 16945113.

Escames G, López LC, Tapias V, Utrilla P, Reiter RJ, Hitos AB, León J, Rodríguez MI, Acuña-Castroviejo D.
Melatonin counteracts inducible mitochondrial nitric oxide synthase-dependent mitochondrial dysfunction in skeletal muscle of septic mice. J Pineal Res. 2006 Jan;40(1):71-8. PubMed PMID: 16313501.

López LC, Escames G, Tapias V, Utrilla P, León J, Acuña-Castroviejo D.
Identification of an inducible nitric oxide synthase in diaphragm mitochondria from septic mice: its relation with mitochondrial dysfunction and prevention by melatonin. Int J Biochem Cell Biol. 2006 Feb;38(2):267-78. Epub 2005 Sep 30. PubMed PMID: 16223598.

Acuña-Castroviejo D, Escames G, López LC, Hitos AB, León J.
Melatonin and nitric oxide: two required antagonists for mitochondrial homeostasis. Endocrine. 2005 Jul;27(2):159-68. Review. PubMed PMID: 16217129.

Escames G, León J, López LC, Acuña-Castroviejo D.
Mechanisms of N-methyl-D-aspartate receptor inhibition by melatonin in the rat striatum. J Neuroendocrinol. 2004 Nov;16(11):929-35. PubMed PMID: 15584934.