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Results: 1 to 20 of 157

1.

Metabolic consequences of NDUFS4 gene deletion in immortalized mouse embryonic fibroblasts.

Valsecchi F, Monge C, Forkink M, de Groof AJ, Benard G, Rossignol R, Swarts HG, van Emst-de Vries SE, Rodenburg RJ, Calvaruso MA, Nijtmans LG, Heeman B, Roestenberg P, Wieringa B, Smeitink JA, Koopman WJ, Willems PH.

Biochim Biophys Acta. 2012 Oct;1817(10):1925-36. doi: 10.1016/j.bbabio.2012.03.006. Epub 2012 Mar 11.

PMID:
22430089
[PubMed - indexed for MEDLINE]
Free Article
2.

Proteomic and metabolomic analyses of mitochondrial complex I-deficient mouse model generated by spontaneous B2 short interspersed nuclear element (SINE) insertion into NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) gene.

Leong DW, Komen JC, Hewitt CA, Arnaud E, McKenzie M, Phipson B, Bahlo M, Laskowski A, Kinkel SA, Davey GM, Heath WR, Voss AK, Zahedi RP, Pitt JJ, Chrast R, Sickmann A, Ryan MT, Smyth GK, Thorburn DR, Scott HS.

J Biol Chem. 2012 Jun 8;287(24):20652-63. doi: 10.1074/jbc.M111.327601. Epub 2012 Apr 25.

PMID:
22535952
[PubMed - indexed for MEDLINE]
Free PMC Article
3.

Primary fibroblasts of NDUFS4(-/-) mice display increased ROS levels and aberrant mitochondrial morphology.

Valsecchi F, Grefte S, Roestenberg P, Joosten-Wagenaars J, Smeitink JA, Willems PH, Koopman WJ.

Mitochondrion. 2013 Sep;13(5):436-43. doi: 10.1016/j.mito.2012.12.001. Epub 2012 Dec 9.

PMID:
23234723
[PubMed - indexed for MEDLINE]
4.

Mitochondrial complex III stabilizes complex I in the absence of NDUFS4 to provide partial activity.

Calvaruso MA, Willems P, van den Brand M, Valsecchi F, Kruse S, Palmiter R, Smeitink J, Nijtmans L.

Hum Mol Genet. 2012 Jan 1;21(1):115-20. doi: 10.1093/hmg/ddr446. Epub 2011 Sep 28.

PMID:
21965299
[PubMed - indexed for MEDLINE]
Free Article
5.

Dysfunctions of cellular oxidative metabolism in patients with mutations in the NDUFS1 and NDUFS4 genes of complex I.

Iuso A, Scacco S, Piccoli C, Bellomo F, Petruzzella V, Trentadue R, Minuto M, Ripoli M, Capitanio N, Zeviani M, Papa S.

J Biol Chem. 2006 Apr 14;281(15):10374-80. Epub 2006 Feb 13.

PMID:
16478720
[PubMed - indexed for MEDLINE]
Free Article
6.

Mice with mitochondrial complex I deficiency develop a fatal encephalomyopathy.

Kruse SE, Watt WC, Marcinek DJ, Kapur RP, Schenkman KA, Palmiter RD.

Cell Metab. 2008 Apr;7(4):312-20. doi: 10.1016/j.cmet.2008.02.004.

PMID:
18396137
[PubMed - indexed for MEDLINE]
Free PMC Article
7.

Human NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology?

Koopman WJ, Verkaart S, Visch HJ, van Emst-de Vries S, Nijtmans LG, Smeitink JA, Willems PH.

Am J Physiol Cell Physiol. 2007 Jul;293(1):C22-9. Epub 2007 Apr 11. Review.

PMID:
17428841
[PubMed - indexed for MEDLINE]
Free Article
8.

Negative regulation of STAT3 protein-mediated cellular respiration by SIRT1 protein.

Bernier M, Paul RK, Martin-Montalvo A, Scheibye-Knudsen M, Song S, He HJ, Armour SM, Hubbard BP, Bohr VA, Wang L, Zong Y, Sinclair DA, de Cabo R.

J Biol Chem. 2011 Jun 3;286(22):19270-9. doi: 10.1074/jbc.M110.200311. Epub 2011 Apr 5.

PMID:
21467030
[PubMed - indexed for MEDLINE]
Free PMC Article
9.

The mitochondrial disease associated protein Ndufaf2 is dispensable for Complex-1 assembly but critical for the regulation of oxidative stress.

Schlehe JS, Journel MS, Taylor KP, Amodeo KD, LaVoie MJ.

Neurobiol Dis. 2013 Oct;58:57-67. doi: 10.1016/j.nbd.2013.05.007. Epub 2013 May 20.

PMID:
23702311
[PubMed - indexed for MEDLINE]
Free PMC Article
10.

Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons.

Sterky FH, Hoffman AF, Milenkovic D, Bao B, Paganelli A, Edgar D, Wibom R, Lupica CR, Olson L, Larsson NG.

Hum Mol Genet. 2012 Mar 1;21(5):1078-89. doi: 10.1093/hmg/ddr537. Epub 2011 Nov 16.

PMID:
22090423
[PubMed - indexed for MEDLINE]
Free PMC Article
11.

Mitochondrial alpha-ketoglutarate dehydrogenase complex generates reactive oxygen species.

Starkov AA, Fiskum G, Chinopoulos C, Lorenzo BJ, Browne SE, Patel MS, Beal MF.

J Neurosci. 2004 Sep 8;24(36):7779-88.

PMID:
15356189
[PubMed - indexed for MEDLINE]
Free Article
12.

Effect of thyroid hormone on mitochondrial properties and oxidative stress in cells from patients with mtDNA defects.

Menzies KJ, Robinson BH, Hood DA.

Am J Physiol Cell Physiol. 2009 Feb;296(2):C355-62. doi: 10.1152/ajpcell.00415.2007. Epub 2008 Nov 26.

PMID:
19036942
[PubMed - indexed for MEDLINE]
Free Article
13.

Cellular and animal models for mitochondrial complex I deficiency: a focus on the NDUFS4 subunit.

Breuer ME, Willems PH, Smeitink JA, Koopman WJ, Nooteboom M.

IUBMB Life. 2013 Mar;65(3):202-8. doi: 10.1002/iub.1127. Epub 2013 Feb 3. Review.

PMID:
23378164
[PubMed - indexed for MEDLINE]
14.

Mitochondrial ND5 gene variation associated with encephalomyopathy and mitochondrial ATP consumption.

McKenzie M, Liolitsa D, Akinshina N, Campanella M, Sisodiya S, Hargreaves I, Nirmalananthan N, Sweeney MG, Abou-Sleiman PM, Wood NW, Hanna MG, Duchen MR.

J Biol Chem. 2007 Dec 21;282(51):36845-52. Epub 2007 Oct 16.

PMID:
17940288
[PubMed - indexed for MEDLINE]
Free Article
15.

Mitochondrial bioenergetics and dynamics interplay in complex I-deficient fibroblasts.

Morán M, Rivera H, Sánchez-Aragó M, Blázquez A, Merinero B, Ugalde C, Arenas J, Cuezva JM, Martín MA.

Biochim Biophys Acta. 2010 May;1802(5):443-53. doi: 10.1016/j.bbadis.2010.02.001. Epub 2010 Feb 11.

PMID:
20153825
[PubMed - indexed for MEDLINE]
Free Article
16.

c-Jun N-terminal kinase regulates mitochondrial bioenergetics by modulating pyruvate dehydrogenase activity in primary cortical neurons.

Zhou Q, Lam PY, Han D, Cadenas E.

J Neurochem. 2008 Jan;104(2):325-35. Epub 2007 Oct 18.

PMID:
17949412
[PubMed - indexed for MEDLINE]
17.

Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV.

Diaz F, Enríquez JA, Moraes CT.

Mol Cell Biol. 2012 Jan;32(2):415-29. doi: 10.1128/MCB.06051-11. Epub 2011 Nov 21.

PMID:
22106410
[PubMed - indexed for MEDLINE]
Free PMC Article
18.

Comparative bioenergetic assessment of transformed cells using a cell energy budget platform.

Zhdanov AV, Favre C, O'Flaherty L, Adam J, O'Connor R, Pollard PJ, Papkovsky DB.

Integr Biol (Camb). 2011 Nov;3(11):1135-42. doi: 10.1039/c1ib00050k. Epub 2011 Oct 17.

PMID:
22005712
[PubMed - indexed for MEDLINE]
19.
20.

Regulation of mitochondrial F(o)F(1)ATPase activity by Sirt3-catalyzed deacetylation and its deficiency in human cells harboring 4977bp deletion of mitochondrial DNA.

Wu YT, Lee HC, Liao CC, Wei YH.

Biochim Biophys Acta. 2013 Jan;1832(1):216-27. doi: 10.1016/j.bbadis.2012.10.002. Epub 2012 Oct 6.

PMID:
23046812
[PubMed - indexed for MEDLINE]
Free Article

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