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Items: 1 to 20 of 101

1.

Survival transcriptome in the coenzyme Q10 deficiency syndrome is acquired by epigenetic modifications: a modelling study for human coenzyme Q10 deficiencies.

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.

BMJ Open. 2013 Mar 25;3(3). pii: e002524. doi: 10.1136/bmjopen-2012-002524.

2.

Effect of Coenzyme Q10 supplementation on mitochondrial electron transport chain activity and mitochondrial oxidative stress in Coenzyme Q10 deficient human neuronal cells.

Duberley KE, Heales SJ, Abramov AY, Chalasani A, Land JM, Rahman S, Hargreaves IP.

Int J Biochem Cell Biol. 2014 May;50:60-3. doi: 10.1016/j.biocel.2014.02.003. Epub 2014 Feb 15.

PMID:
24534273
3.

Pathomechanisms in coenzyme q10-deficient human fibroblasts.

López LC, Luna-Sánchez M, García-Corzo L, Quinzii CM, Hirano M.

Mol Syndromol. 2014 Jul;5(3-4):163-9. doi: 10.1159/000360494.

4.

Secondary coenzyme Q10 deficiency and oxidative stress in cultured fibroblasts from patients with riboflavin responsive multiple Acyl-CoA dehydrogenation deficiency.

Cornelius N, Byron C, Hargreaves I, Guerra PF, Furdek AK, Land J, Radford WW, Frerman F, Corydon TJ, Gregersen N, Olsen RK.

Hum Mol Genet. 2013 Oct 1;22(19):3819-27. doi: 10.1093/hmg/ddt232. Epub 2013 May 31.

5.

Genetics of coenzyme q10 deficiency.

Doimo M, Desbats MA, Cerqua C, Cassina M, Trevisson E, Salviati L.

Mol Syndromol. 2014 Jul;5(3-4):156-62. doi: 10.1159/000362826.

6.

Primary coenzyme Q10 (CoQ 10) deficiencies and related nephropathies.

Ozaltin F.

Pediatr Nephrol. 2014 Jun;29(6):961-9. doi: 10.1007/s00467-013-2482-z. Epub 2013 Jun 5. Review.

PMID:
23736673
7.

Clinical presentations of coenzyme q10 deficiency syndrome.

Quinzii CM, Emmanuele V, Hirano M.

Mol Syndromol. 2014 Jul;5(3-4):141-6. doi: 10.1159/000360490.

8.

Coenzyme q10 therapy.

Garrido-Maraver J, Cordero MD, Oropesa-Ávila M, Fernández Vega A, de la Mata M, Delgado Pavón A, de Miguel M, Pérez Calero C, Villanueva Paz M, Cotán D, Sánchez-Alcázar JA.

Mol Syndromol. 2014 Jul;5(3-4):187-97. doi: 10.1159/000360101.

9.

Coenzyme Q10 defects may be associated with a deficiency of Q10-independent mitochondrial respiratory chain complexes.

Fragaki K, Chaussenot A, Benoist JF, Ait-El-Mkadem S, Bannwarth S, Rouzier C, Cochaud C, Paquis-Flucklinger V.

Biol Res. 2016 Jan 8;49:4. doi: 10.1186/s40659-015-0065-0.

10.

Decreased Coenzyme Q10 Levels in Multiple System Atrophy Cerebellum.

Barca E, Kleiner G, Tang G, Ziosi M, Tadesse S, Masliah E, Louis ED, Faust P, Kang UJ, Torres J, Cortes EP, Vonsattel JP, Kuo SH, Quinzii CM.

J Neuropathol Exp Neurol. 2016 Jul;75(7):663-72. doi: 10.1093/jnen/nlw037. Epub 2016 May 27.

PMID:
27235405
11.

Bioenergetic and antioxidant properties of coenzyme Q10: recent developments.

Littarru GP, Tiano L.

Mol Biotechnol. 2007 Sep;37(1):31-7. Review.

PMID:
17914161
12.

Primary and secondary coenzyme Q10 deficiency: the role of therapeutic supplementation.

Potgieter M, Pretorius E, Pepper MS.

Nutr Rev. 2013 Mar;71(3):180-8. doi: 10.1111/nure.12011. Epub 2013 Jan 30. Review.

PMID:
23452285
13.

Familial cerebellar ataxia with muscle coenzyme Q10 deficiency.

Musumeci O, Naini A, Slonim AE, Skavin N, Hadjigeorgiou GL, Krawiecki N, Weissman BM, Tsao CY, Mendell JR, Shanske S, De Vivo DC, Hirano M, DiMauro S.

Neurology. 2001 Apr 10;56(7):849-55.

PMID:
11294920
14.

Treatment effect of coenzyme Q(10) and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease.

Matalonga L, Arias A, Coll MJ, Garcia-Villoria J, Gort L, Ribes A.

J Inherit Metab Dis. 2014 May;37(3):439-46. doi: 10.1007/s10545-013-9668-1. Epub 2013 Dec 18.

PMID:
24347096
15.

Association between genetic variants in the Coenzyme Q10 metabolism and Coenzyme Q10 status in humans.

Fischer A, Schmelzer C, Rimbach G, Niklowitz P, Menke T, Döring F.

BMC Res Notes. 2011 Jul 21;4:245. doi: 10.1186/1756-0500-4-245.

16.

Human CoQ10 deficiencies.

Quinzii CM, López LC, Naini A, DiMauro S, Hirano M.

Biofactors. 2008;32(1-4):113-8.

17.

Ubiquinol-10 ameliorates mitochondrial encephalopathy associated with CoQ deficiency.

García-Corzo L, Luna-Sánchez M, Doerrier C, Ortiz F, Escames G, Acuña-Castroviejo D, López LC.

Biochim Biophys Acta. 2014 Jul;1842(7):893-901. doi: 10.1016/j.bbadis.2014.02.008. Epub 2014 Feb 24.

18.

Primary coenzyme Q10 deficiency presenting as fatal neonatal multiorgan failure.

Desbats MA, Vetro A, Limongelli I, Lunardi G, Casarin A, Doimo M, Spinazzi M, Angelini C, Cenacchi G, Burlina A, Rodriguez Hernandez MA, Chiandetti L, Clementi M, Trevisson E, Navas P, Zuffardi O, Salviati L.

Eur J Hum Genet. 2015 Sep;23(9):1254-8. doi: 10.1038/ejhg.2014.277. Epub 2015 Jan 7.

19.

Clinical applications of coenzyme Q10.

Garrido-Maraver J, Cordero MD, Oropesa-Avila M, Vega AF, de la Mata M, Pavon AD, Alcocer-Gomez E, Calero CP, Paz MV, Alanis M, de Lavera I, Cotan D, Sanchez-Alcazar JA.

Front Biosci (Landmark Ed). 2014 Jan 1;19:619-33. Review.

PMID:
24389208
20.

Cellular redox activity of coenzyme Q10: effect of CoQ10 supplementation on human skeletal muscle.

Linnane AW, Kopsidas G, Zhang C, Yarovaya N, Kovalenko S, Papakostopoulos P, Eastwood H, Graves S, Richardson M.

Free Radic Res. 2002 Apr;36(4):445-53.

PMID:
12069109
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