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Items: 1 to 20 of 109

1.

Modes of metabolic compensation during mitochondrial disease using the Drosophila model of ATP6 dysfunction.

Celotto AM, Chiu WK, Van Voorhies W, Palladino MJ.

PLoS One. 2011;6(10):e25823. doi: 10.1371/journal.pone.0025823. Epub 2011 Oct 3.

2.

Mitochondrial encephalomyopathy in Drosophila.

Celotto AM, Frank AC, McGrath SW, Fergestad T, Van Voorhies WA, Buttle KF, Mannella CA, Palladino MJ.

J Neurosci. 2006 Jan 18;26(3):810-20.

3.

Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis.

Menzies FM, Cookson MR, Taylor RW, Turnbull DM, Chrzanowska-Lightowlers ZM, Dong L, Figlewicz DA, Shaw PJ.

Brain. 2002 Jul;125(Pt 7):1522-33.

4.

Consequences of the pathogenic T9176C mutation of human mitochondrial DNA on yeast mitochondrial ATP synthase.

Kucharczyk R, Ezkurdia N, Couplan E, Procaccio V, Ackerman SH, Blondel M, di Rago JP.

Biochim Biophys Acta. 2010 Jun-Jul;1797(6-7):1105-12. doi: 10.1016/j.bbabio.2009.12.022. Epub 2010 Jan 4.

5.

A Drosophila model of mitochondrial disease caused by a complex I mutation that uncouples proton pumping from electron transfer.

Burman JL, Itsara LS, Kayser EB, Suthammarak W, Wang AM, Kaeberlein M, Sedensky MM, Morgan PG, Pallanck LJ.

Dis Model Mech. 2014 Oct;7(10):1165-74. doi: 10.1242/dmm.015321. Epub 2014 Aug 1.

6.

New insights into the bioenergetics of mitochondrial disorders using intracellular ATP reporters.

Gajewski CD, Yang L, Schon EA, Manfredi G.

Mol Biol Cell. 2003 Sep;14(9):3628-35. Epub 2003 Jun 27.

7.

Mitochondrial DNA background modifies the bioenergetics of NARP/MILS ATP6 mutant cells.

D'Aurelio M, Vives-Bauza C, Davidson MM, Manfredi G.

Hum Mol Genet. 2010 Jan 15;19(2):374-86. doi: 10.1093/hmg/ddp503. Epub 2009 Oct 29.

8.

Cardiac insulin-resistance and decreased mitochondrial energy production precede the development of systolic heart failure after pressure-overload hypertrophy.

Zhang L, Jaswal JS, Ussher JR, Sankaralingam S, Wagg C, Zaugg M, Lopaschuk GD.

Circ Heart Fail. 2013 Sep 1;6(5):1039-48. doi: 10.1161/CIRCHEARTFAILURE.112.000228. Epub 2013 Jul 16.

9.

Development of a human mitochondrial oligonucleotide microarray (h-MitoArray) and gene expression analysis of fibroblast cell lines from 13 patients with isolated F1Fo ATP synthase deficiency.

Cízková A, Stránecký V, Ivánek R, Hartmannová H, Nosková L, Piherová L, Tesarová M, Hansíková H, Honzík T, Zeman J, Divina P, Potocká A, Paul J, Sperl W, Mayr JA, Seneca S, Houstĕk J, Kmoch S.

BMC Genomics. 2008 Jan 25;9:38. doi: 10.1186/1471-2164-9-38.

10.

A metabolic model of the mitochondrion and its use in modelling diseases of the tricarboxylic acid cycle.

Smith AC, Robinson AJ.

BMC Syst Biol. 2011 Jun 29;5:102. doi: 10.1186/1752-0509-5-102.

11.

Diminished synthesis of subunit a (ATP6) and altered function of ATP synthase and cytochrome c oxidase due to the mtDNA 2 bp microdeletion of TA at positions 9205 and 9206.

Jesina P, Tesarová M, Fornůsková D, Vojtísková A, Pecina P, Kaplanová V, Hansíková H, Zeman J, Houstek J.

Biochem J. 2004 Nov 1;383(Pt. 3):561-71.

12.

Glutathione s-transferase omega 1 activity is sufficient to suppress neurodegeneration in a Drosophila model of Parkinson disease.

Kim K, Kim SH, Kim J, Kim H, Yim J.

J Biol Chem. 2012 Feb 24;287(9):6628-41. doi: 10.1074/jbc.M111.291179. Epub 2012 Jan 4.

13.

Mitochondrial transcription factor B2 is essential for metabolic function in Drosophila melanogaster development.

Adán C, Matsushima Y, Hernández-Sierra R, Marco-Ferreres R, Fernández-Moreno MA, González-Vioque E, Calleja M, Aragón JJ, Kaguni LS, Garesse R.

J Biol Chem. 2008 May 2;283(18):12333-42. doi: 10.1074/jbc.M801342200. Epub 2008 Feb 28.

14.

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
15.

Modeling mitochondrial encephalomyopathy in Drosophila.

Palladino MJ.

Neurobiol Dis. 2010 Oct;40(1):40-5. doi: 10.1016/j.nbd.2010.05.009. Epub 2010 May 21. Review.

16.

Allotopic expression of ATP6 in the mouse as a transgenic model of mitochondrial disease.

Dunn DA, Pinkert CA.

Methods Mol Biol. 2015;1265:255-69. doi: 10.1007/978-1-4939-2288-8_18.

PMID:
25634280
17.

Reactive oxygen species act remotely to cause synapse loss in a Drosophila model of developmental mitochondrial encephalopathy.

Mast JD, Tomalty KM, Vogel H, Clandinin TR.

Development. 2008 Aug;135(15):2669-79. doi: 10.1242/dev.020644. Epub 2008 Jul 3.

18.

Mitochondrial diseases and genetic defects of ATP synthase.

Houstek J, Pícková A, Vojtísková A, Mrácek T, Pecina P, Jesina P.

Biochim Biophys Acta. 2006 Sep-Oct;1757(9-10):1400-5. Epub 2006 Apr 19. Review.

19.

Post-transcriptional regulation of the mitochondrial H(+)-ATP synthase: a key regulator of the metabolic phenotype in cancer.

Willers IM, Cuezva JM.

Biochim Biophys Acta. 2011 Jun;1807(6):543-51. doi: 10.1016/j.bbabio.2010.10.016. Epub 2010 Oct 27. Review.

20.

Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins.

Schwab MA, Sauer SW, Okun JG, Nijtmans LG, Rodenburg RJ, van den Heuvel LP, Dröse S, Brandt U, Hoffmann GF, Ter Laak H, Kölker S, Smeitink JA.

Biochem J. 2006 Aug 15;398(1):107-12.

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