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Results: 1 to 20 of 100

1.

Dysfunctional Coq9 protein causes predominant encephalomyopathy associated with CoQ deficiency.

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.

Hum Mol Genet. 2013 Mar 15;22(6):1233-48. doi: 10.1093/hmg/dds530. Epub 2012 Dec 18.

PMID:
23255162
[PubMed - indexed for MEDLINE]
Free Article
2.

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.

PMID:
24576561
[PubMed - indexed for MEDLINE]
3.

Treatment of CoQ(10) deficient fibroblasts with ubiquinone, CoQ analogs, and vitamin C: time- and compound-dependent effects.

López LC, Quinzii CM, Area E, Naini A, Rahman S, Schuelke M, Salviati L, Dimauro S, Hirano M.

PLoS One. 2010 Jul 30;5(7):e11897. doi: 10.1371/journal.pone.0011897.

PMID:
20689595
[PubMed - indexed for MEDLINE]
Free PMC Article
4.

Human neuronal coenzyme Q10 deficiency results in global loss of mitochondrial respiratory chain activity, increased mitochondrial oxidative stress and reversal of ATP synthase activity: implications for pathogenesis and treatment.

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

J Inherit Metab Dis. 2013 Jan;36(1):63-73. doi: 10.1007/s10545-012-9511-0. Epub 2012 Jul 6.

PMID:
22767283
[PubMed - indexed for MEDLINE]
5.

Tissue-specific oxidative stress and loss of mitochondria in CoQ-deficient Pdss2 mutant mice.

Quinzii CM, Garone C, Emmanuele V, Tadesse S, Krishna S, Dorado B, Hirano M.

FASEB J. 2013 Feb;27(2):612-21. doi: 10.1096/fj.12-209361. Epub 2012 Nov 12.

PMID:
23150520
[PubMed - indexed for MEDLINE]
Free PMC Article
6.

Primary coenzyme Q deficiency in Pdss2 mutant mice causes isolated renal disease.

Peng M, Falk MJ, Haase VH, King R, Polyak E, Selak M, Yudkoff M, Hancock WW, Meade R, Saiki R, Lunceford AL, Clarke CF, Gasser DL.

PLoS Genet. 2008 Apr 25;4(4):e1000061. doi: 10.1371/journal.pgen.1000061.

PMID:
18437205
[PubMed - indexed for MEDLINE]
Free PMC Article
7.

Coenzyme Q and mitochondrial disease.

Quinzii CM, Hirano M.

Dev Disabil Res Rev. 2010 Jun;16(2):183-8. doi: 10.1002/ddrr.108. Review.

PMID:
20818733
[PubMed - indexed for MEDLINE]
Free PMC Article
8.

Coenzyme Q deficiency triggers mitochondria degradation by mitophagy.

Rodríguez-Hernández A, Cordero MD, Salviati L, Artuch R, Pineda M, Briones P, Gómez Izquierdo L, Cotán D, Navas P, Sánchez-Alcázar JA.

Autophagy. 2009 Jan;5(1):19-32.

PMID:
19115482
[PubMed - indexed for MEDLINE]
Free Article
9.

Muscle coenzyme Q: a potential test for mitochondrial activity and redox status.

Miles L, Miles MV, Tang PH, Horn PS, Wong BL, DeGrauw TJ, Morehart PJ, Bove KE.

Pediatr Neurol. 2005 May;32(5):318-24.

PMID:
15866432
[PubMed - indexed for MEDLINE]
10.

Changes in the content and intracellular distribution of coenzyme Q homologs in rabbit liver during growth.

Matsura T, Yamada K, Kawasaki T.

Biochim Biophys Acta. 1991 Jun 3;1083(3):277-82.

PMID:
2049392
[PubMed - indexed for MEDLINE]
11.

A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency.

Quinzii C, Naini A, Salviati L, Trevisson E, Navas P, Dimauro S, Hirano M.

Am J Hum Genet. 2006 Feb;78(2):345-9. Epub 2005 Dec 22.

PMID:
16400613
[PubMed - indexed for MEDLINE]
Free PMC Article
12.

Update on clinical aspects and treatment of selected vitamin-responsive disorders II (riboflavin and CoQ 10).

Horvath R.

J Inherit Metab Dis. 2012 Jul;35(4):679-87. doi: 10.1007/s10545-011-9434-1. Epub 2012 Jan 10. Review.

PMID:
22231380
[PubMed - indexed for MEDLINE]
13.

Mitochondrial coenzyme Q content and aging.

Lass A, Kwong L, Sohal RS.

Biofactors. 1999;9(2-4):199-205.

PMID:
10416032
[PubMed - indexed for MEDLINE]
14.

Coenzyme Q supplementation or over-expression of the yeast Coq8 putative kinase stabilizes multi-subunit Coq polypeptide complexes in yeast coq null mutants.

He CH, Xie LX, Allan CM, Tran UC, Clarke CF.

Biochim Biophys Acta. 2014 Apr 4;1841(4):630-44. doi: 10.1016/j.bbalip.2013.12.017. Epub 2014 Jan 7.

PMID:
24406904
[PubMed - indexed for MEDLINE]
15.

Predominant cerebellar volume loss as a neuroradiologic feature of pediatric respiratory chain defects.

Scaglia F, Wong LJ, Vladutiu GD, Hunter JV.

AJNR Am J Neuroradiol. 2005 Aug;26(7):1675-80. Erratum in: AJNR Am J Neuroradiol. 2005 Sep;26(8):2165.

PMID:
16091512
[PubMed - indexed for MEDLINE]
Free Article
16.

CoQ(10) deficiencies and MNGIE: two treatable mitochondrial disorders.

Hirano M, Garone C, Quinzii CM.

Biochim Biophys Acta. 2012 May;1820(5):625-31. doi: 10.1016/j.bbagen.2012.01.006. Epub 2012 Jan 18. Review.

PMID:
22274133
[PubMed - indexed for MEDLINE]
Free PMC Article
17.

coq7/clk-1 regulates mitochondrial respiration and the generation of reactive oxygen species via coenzyme Q.

Nakai D, Shimizu T, Nojiri H, Uchiyama S, Koike H, Takahashi M, Hirokawa K, Shirasawa T.

Aging Cell. 2004 Oct;3(5):273-81.

PMID:
15379851
[PubMed - indexed for MEDLINE]
18.

Primary and secondary CoQ(10) deficiencies in humans.

Quinzii CM, Hirano M.

Biofactors. 2011 Sep-Oct;37(5):361-5. doi: 10.1002/biof.155. Epub 2011 Oct 11. Review.

PMID:
21990098
[PubMed - indexed for MEDLINE]
Free PMC Article
19.

Altered redox status of coenzyme Q9 reflects mitochondrial electron transport chain deficiencies in Caenorhabditis elegans.

Vasta V, Sedensky M, Morgan P, Hahn SH.

Mitochondrion. 2011 Jan;11(1):136-8. doi: 10.1016/j.mito.2010.09.002. Epub 2010 Sep 16.

PMID:
20849980
[PubMed - indexed for MEDLINE]
20.

Respiratory chain dysfunction and oxidative stress correlate with severity of primary CoQ10 deficiency.

Quinzii CM, López LC, Von-Moltke J, Naini A, Krishna S, Schuelke M, Salviati L, Navas P, DiMauro S, Hirano M.

FASEB J. 2008 Jun;22(6):1874-85. doi: 10.1096/fj.07-100149. Epub 2008 Jan 29.

PMID:
18230681
[PubMed - indexed for MEDLINE]
Free PMC Article

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