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Items: 32

1.

Inadequate control of thyroid hormones sensitizes to hepatocarcinogenesis and unhealthy aging.

López-Noriega L, Capilla-González V, Cobo-Vuilleumier N, Martin-Vazquez E, Lorenzo PI, Martinez-Force E, Soriano-Navarro M, García-Fernández M, Romero-Zerbo SY, Bermúdez-Silva FJ, Díaz-Contreras I, Sánchez-Cuesta A, Santos-Ocaña C, Hmadcha A, Soria B, Martín F, Gauthier BR, Martin-Montalvo A.

Aging (Albany NY). 2019 Sep 13;11(18):7746-7779. doi: 10.18632/aging.102285. Epub 2019 Sep 13.

2.

ADCK2 Haploinsufficiency Reduces Mitochondrial Lipid Oxidation and Causes Myopathy Associated with CoQ Deficiency.

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.

J Clin Med. 2019 Sep 2;8(9). pii: E1374. doi: 10.3390/jcm8091374.

3.

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.

Biochim Biophys Acta Bioenerg. 2018 Nov;1859(11):1235-1248. doi: 10.1016/j.bbabio.2018.09.369. Epub 2018 Sep 26.

PMID:
30267671
4.

Molecular diagnosis of coenzyme Q10 deficiency: an update.

Yubero D, Montero R, Santos-Ocaña C, Salviati L, Navas P, Artuch R.

Expert Rev Mol Diagn. 2018 Jun;18(6):491-498. doi: 10.1080/14737159.2018.1478290. Epub 2018 May 30. Review.

PMID:
29781757
5.

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.

Stem Cells. 2017 Jul;35(7):1687-1703. doi: 10.1002/stem.2634. Epub 2017 May 23.

6.

Generation, genome edition and characterization of iPSC lines from a patient with coenzyme Q10 deficiency harboring a heterozygous mutation in COQ4 gene.

Romero-Moya D, Castaño J, Santos-Ocaña C, Navas P, Menendez P.

Stem Cell Res. 2017 Oct;24:144-147. doi: 10.1016/j.scr.2016.09.007. Epub 2016 Sep 16.

7.

Balanced CoQ6 biosynthesis is required for lifespan and mitophagy in yeast.

González-Mariscal I, Martín-Montalvo A, Ojeda-González C, Rodríguez-Eguren A, Gutiérrez-Ríos P, Navas P, Santos-Ocaña C.

Microb Cell. 2017 Feb 3;4(2):38-51. doi: 10.15698/mic2017.02.556.

8.

Structural basis of mitochondrial dysfunction in response to cytochrome c phosphorylation at tyrosine 48.

Moreno-Beltrán B, Guerra-Castellano A, Díaz-Quintana A, Del Conte R, García-Mauriño SM, Díaz-Moreno S, González-Arzola K, Santos-Ocaña C, Velázquez-Campoy A, De la Rosa MA, Turano P, Díaz-Moreno I.

Proc Natl Acad Sci U S A. 2017 Apr 11;114(15):E3041-E3050. doi: 10.1073/pnas.1618008114. Epub 2017 Mar 27.

9.

Molecular diagnosis of coenzyme Q10 deficiency.

Yubero D, Montero R, Armstrong J, Espinós C, Palau F, Santos-Ocaña C, Salviati L, Navas P, Artuch R.

Expert Rev Mol Diagn. 2015;15(8):1049-59. doi: 10.1586/14737159.2015.1062727. Epub 2015 Jul 4. Review.

PMID:
26144946
10.

Mitochondrial responsibility in ageing process: innocent, suspect or guilty.

López-Lluch G, Santos-Ocaña C, Sánchez-Alcázar JA, Fernández-Ayala DJ, Asencio-Salcedo C, Rodríguez-Aguilera JC, Navas P.

Biogerontology. 2015 Oct;16(5):599-620. doi: 10.1007/s10522-015-9585-9. Epub 2015 Jun 24. Review.

PMID:
26105157
11.

Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis.

Nguyen TP, Casarin A, Desbats MA, Doimo M, Trevisson E, Santos-Ocaña C, Navas P, Clarke CF, Salviati L.

Biochim Biophys Acta. 2014 Nov;1841(11):1628-38. doi: 10.1016/j.bbalip.2014.08.007. Epub 2014 Aug 23.

12.

The regulation of coenzyme q biosynthesis in eukaryotic cells: all that yeast can tell us.

González-Mariscal I, García-Testón E, Padilla S, Martín-Montalvo A, Pomares Viciana T, Vazquez-Fonseca L, Gandolfo Domínguez P, Santos-Ocaña C.

Mol Syndromol. 2014 Jul;5(3-4):107-18. doi: 10.1159/000362897.

13.

Regulation of coenzyme Q biosynthesis in yeast: a new complex in the block.

González-Mariscal I, García-Testón E, Padilla S, Martín-Montalvo A, Pomares-Viciana T, Vazquez-Fonseca L, Gandolfo-Domínguez P, Santos-Ocaña C.

IUBMB Life. 2014 Feb;66(2):63-70. doi: 10.1002/iub.1243. Epub 2014 Jan 27. Review.

14.

Effect of vanillic acid on COQ6 mutants identified in patients with coenzyme Q10 deficiency.

Doimo M, Trevisson E, Airik R, Bergdoll M, Santos-Ocaña C, Hildebrandt F, Navas P, Pierrel F, Salviati L.

Biochim Biophys Acta. 2014 Jan;1842(1):1-6. doi: 10.1016/j.bbadis.2013.10.007. Epub 2013 Oct 18.

15.

The phosphatase Ptc7 induces coenzyme Q biosynthesis by activating the hydroxylase Coq7 in yeast.

Martín-Montalvo A, González-Mariscal I, Pomares-Viciana T, Padilla-López S, Ballesteros M, Vazquez-Fonseca L, Gandolfo P, Brautigan DL, Navas P, Santos-Ocaña C.

J Biol Chem. 2013 Sep 27;288(39):28126-37. doi: 10.1074/jbc.M113.474494. Epub 2013 Aug 12.

16.

Haploinsufficiency of COQ4 causes coenzyme Q10 deficiency.

Salviati L, Trevisson E, Rodriguez Hernandez MA, Casarin A, Pertegato V, Doimo M, Cassina M, Agosto C, Desbats MA, Sartori G, Sacconi S, Memo L, Zuffardi O, Artuch R, Quinzii C, Dimauro S, Hirano M, Santos-Ocaña C, Navas P.

J Med Genet. 2012 Mar;49(3):187-91. doi: 10.1136/jmedgenet-2011-100394.

17.

Respiratory-induced coenzyme Q biosynthesis is regulated by a phosphorylation cycle of Cat5p/Coq7p.

Martín-Montalvo A, González-Mariscal I, Padilla S, Ballesteros M, Brautigan DL, Navas P, Santos-Ocaña C.

Biochem J. 2011 Nov 15;440(1):107-14. doi: 10.1042/BJ20101422.

PMID:
21812761
18.

COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness.

Heeringa SF, Chernin G, Chaki M, Zhou W, Sloan AJ, Ji Z, Xie LX, Salviati L, Hurd TW, Vega-Warner V, Killen PD, Raphael Y, Ashraf S, Ovunc B, Schoeb DS, McLaughlin HM, Airik R, Vlangos CN, Gbadegesin R, Hinkes B, Saisawat P, Trevisson E, Doimo M, Casarin A, Pertegato V, Giorgi G, Prokisch H, Rötig A, Nürnberg G, Becker C, Wang S, Ozaltin F, Topaloglu R, Bakkaloglu A, Bakkaloglu SA, Müller D, Beissert A, Mir S, Berdeli A, Varpizen S, Zenker M, Matejas V, Santos-Ocaña C, Navas P, Kusakabe T, Kispert A, Akman S, Soliman NA, Krick S, Mundel P, Reiser J, Nürnberg P, Clarke CF, Wiggins RC, Faul C, Hildebrandt F.

J Clin Invest. 2011 May;121(5):2013-24. doi: 10.1172/JCI45693. Epub 2011 Apr 11.

19.

Is coenzyme Q a key factor in aging?

López-Lluch G, Rodríguez-Aguilera JC, Santos-Ocaña C, Navas P.

Mech Ageing Dev. 2010 Apr;131(4):225-35. doi: 10.1016/j.mad.2010.02.003. Epub 2010 Mar 1. Review.

PMID:
20193705
20.

Genetic evidence for the requirement of the endocytic pathway in the uptake of coenzyme Q6 in Saccharomyces cerevisiae.

Padilla-López S, Jiménez-Hidalgo M, Martín-Montalvo A, Clarke CF, Navas P, Santos-Ocaña C.

Biochim Biophys Acta. 2009 Jun;1788(6):1238-48. doi: 10.1016/j.bbamem.2009.03.018. Epub 2009 Apr 2.

21.

NQR1 controls lifespan by regulating the promotion of respiratory metabolism in yeast.

Jiménez-Hidalgo M, Santos-Ocaña C, Padilla S, Villalba JM, López-Lluch G, Martín-Montalvo A, Minor RK, Sinclair DA, de Cabo R, Navas P.

Aging Cell. 2009 Apr;8(2):140-51. doi: 10.1111/j.1474-9726.2009.00461.x. Epub 2009 Feb 23.

22.

Hydroxylation of demethoxy-Q6 constitutes a control point in yeast coenzyme Q6 biosynthesis.

Padilla S, Tran UC, Jiménez-Hidalgo M, López-Martín JM, Martín-Montalvo A, Clarke CF, Navas P, Santos-Ocaña C.

Cell Mol Life Sci. 2009 Jan;66(1):173-86. doi: 10.1007/s00018-008-8547-7.

23.

Missense mutation of the COQ2 gene causes defects of bioenergetics and de novo pyrimidine synthesis.

López-Martín JM, Salviati L, Trevisson E, Montini G, DiMauro S, Quinzii C, Hirano M, Rodriguez-Hernandez A, Cordero MD, Sánchez-Alcázar JA, Santos-Ocaña C, Navas P.

Hum Mol Genet. 2007 May 1;16(9):1091-7. Epub 2007 Mar 20.

24.

Coenzyme Q is irreplaceable by demethoxy-coenzyme Q in plasma membrane of Caenorhabditis elegans.

Arroyo A, Santos-Ocaña C, Ruiz-Ferrer M, Padilla S, Gavilán A, Rodríguez-Aguilera JC, Navas P.

FEBS Lett. 2006 Mar 20;580(7):1740-6. Epub 2006 Feb 20.

25.

Demethoxy-Q, an intermediate of coenzyme Q biosynthesis, fails to support respiration in Saccharomyces cerevisiae and lacks antioxidant activity.

Padilla S, Jonassen T, Jiménez-Hidalgo MA, Fernández-Ayala DJ, López-Lluch G, Marbois B, Navas P, Clarke CF, Santos-Ocaña C.

J Biol Chem. 2004 Jun 18;279(25):25995-6004. Epub 2004 Apr 12.

26.

Stabilization of extracellular ascorbate mediated by coenzyme Q transmembrane electron transport.

Arroyo A, Rodríguez-Aguilera JC, Santos-Ocaña C, Villalba JM, Navas P.

Methods Enzymol. 2004;378:207-17. Review. No abstract available.

PMID:
15038971
27.

Uptake of exogenous coenzyme Q and transport to mitochondria is required for bc1 complex stability in yeast coq mutants.

Santos-Ocaña C, Do TQ, Padilla S, Navas P, Clarke CF.

J Biol Chem. 2002 Mar 29;277(13):10973-81. Epub 2002 Jan 11.

28.

Plasma membrane redox system protects cells against oxidative stress.

Rodríguez-Aguilera JC, López-Lluch G, Santos-Ocaña C, Villalba JM, Gómez-Díaz C, Navas P.

Redox Rep. 2000;5(2-3):148-50. No abstract available.

PMID:
10939299
29.
30.

Genetic evidence for coenzyme Q requirement in plasma membrane electron transport.

Santos-Ocaña C, Villalba JM, Córdoba F, Padilla S, Crane FL, Clarke CF, Navas P.

J Bioenerg Biomembr. 1998 Oct;30(5):465-75.

PMID:
9932649
31.
32.

Extracellular ascorbate stabilization as a result of transplasma electron transfer in Saccharomyces cerevisiae.

Santos-Ocaña C, Navas P, Crane FL, Córdoba F.

J Bioenerg Biomembr. 1995 Dec;27(6):597-603.

PMID:
8746846

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