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Items: 1 to 20 of 206

1.
Caenorhabditis elegans UCP4 Protein Controls Complex II-mediated Oxidative Phosphorylation through Succinate Transport
Matthew Pfeiffer, Ernst-Bernhard Kayzer, Xianmei Yang, Ellen Abramson, M. Alexander Kenaston, Cory U. Lago, Herng-Hsiang Lo, Margaret M. Sedensky, Adam Lunceford, Catherine F. Clarke, Sarah J. Wu, Chris McLeod, Toren Finkel, Philip G. Morgan, Edward M. Mills
J Biol Chem. 2011 Oct 28; 286(43): 37712–37720. Published online 2011 Aug 23. doi: 10.1074/jbc.M111.271452
PMCID:
PMC3199514
AbstractArticlePDF–1.5M
2.
Refractory epilepsy and mitochondrial dysfunction due to GM3 synthase deficiency
Konstantina Fragaki, Samira Ait-El-Mkadem, Annabelle Chaussenot, Catherine Gire, Raymond Mengual, Laurent Bonesso, Marie Bénéteau, Jean-Ehrland Ricci, Valérie Desquiret-Dumas, Vincent Procaccio, Agnès Rötig, Véronique Paquis-Flucklinger
Eur J Hum Genet. 2013 May; 21(5): 528–534. Published online 2012 Sep 19. doi: 10.1038/ejhg.2012.202
PMCID:
PMC3641379
AbstractArticlePDF–917K
3.
Mice Deficient in the Respiratory Chain Gene Cox6a2 Are Protected against High-Fat Diet-Induced Obesity and Insulin Resistance
Roel Quintens, Sarvjeet Singh, Katleen Lemaire, Katrien De Bock, Mikaela Granvik, Anica Schraenen, Irene Olga Cornelia Maria Vroegrijk, Veronica Costa, Pieter Van Noten, Dennis Lambrechts, Stefan Lehnert, Leentje Van Lommel, Lieven Thorrez, Geoffroy De Faudeur, Johannes Anthonius Romijn, John Michael Shelton, Luca Scorrano, Henri Roger Lijnen, Peter Jacobus Voshol, Peter Carmeliet, Pradeep Puthenveetil Abraham Mammen, Frans Schuit
PLoS One. 2013; 8(2): e56719. Published online 2013 Feb 27. doi: 10.1371/journal.pone.0056719
PMCID:
PMC3584060
AbstractArticlePDF–1.0M
4.
Phosphatidylethanolamine Deficiency in Mammalian Mitochondria Impairs Oxidative Phosphorylation and Alters Mitochondrial Morphology
Guergana Tasseva, Helin Daniel Bai, Magdalena Davidescu, Alois Haromy, Evangelos Michelakis, Jean E. Vance
J Biol Chem. 2013 Feb 8; 288(6): 4158–4173. Published online 2012 Dec 18. doi: 10.1074/jbc.M112.434183
PMCID:
PMC3567666
AbstractArticlePDF–4.3M
5.
A defect in the mitochondrial complex III, but not complex IV, triggers early ROS-dependent damage in defined brain regions
Francisca Diaz, Sofia Garcia, Kyle R. Padgett, Carlos T. Moraes
Hum Mol Genet. 2012 Dec 1; 21(23): 5066–5077. Published online 2012 Aug 21. doi: 10.1093/hmg/dds350
PMCID:
PMC3490513
AbstractArticlePDF–528K
6.
Mutation in PNPT1, which Encodes a Polyribonucleotide Nucleotidyltransferase, Impairs RNA Import into Mitochondria and Causes Respiratory-Chain Deficiency
Vanessa Vedrenne, Ali Gowher, Pascale De Lonlay, Patrick Nitschke, Valérie Serre, Nathalie Boddaert, Cecilia Altuzarra, Anne-Marie Mager-Heckel, Florence Chretien, Nina Entelis, Arnold Munnich, Ivan Tarassov, Agnès Rötig
Am J Hum Genet. 2012 Nov 2; 91(5): 912–918. doi: 10.1016/j.ajhg.2012.09.001
PMCID:
PMC3487136
AbstractArticlePDF–748K
7.
Restoring de novo Coenzyme Q biosynthesis in Caenorhabditis elegans coq-3 mutants yields profound rescue compared to exogenous Coenzyme Q supplementation
Fernando Gomez, Ryoichi Saiki, Randall Chin, Chandra Srinivasan, Catherine F. Clarke
Gene. Author manuscript; available in PMC 2013 Sep 10.
Published in final edited form as: Gene. 2012 Sep 10; 506(1): 106–116. Published online 2012 Jun 23. doi: 10.1016/j.gene.2012.06.023
PMCID:
PMC3437764
AbstractArticlePDF–1.5M
8.
Natural disease course and genotype-phenotype correlations in Complex I deficiency caused by nuclear gene defects: what we learned from 130 cases
S. Koene, R. J. Rodenburg, M. S. van der Knaap, M. A. A. P. Willemsen, W. Sperl, V. Laugel, E. Ostergaard, M. Tarnopolsky, M. A. Martin, V. Nesbitt, J. Fletcher, S. Edvardson, V. Procaccio, A. Slama, L. P. W. J. van den Heuvel, J. A. M. Smeitink
J Inherit Metab Dis. 2012; 35(5): 737–747. Published online 2012 May 30. doi: 10.1007/s10545-012-9492-z
PMCID:
PMC3432203
AbstractArticlePDF–232K
9.
Mutations in the mitochondrial tRNASer(AGY) gene are associated with deafness, retinal degeneration, myopathy and epilepsy
Helen AL Tuppen, Karin Naess, Nancy G Kennaway, Mazhor Al-Dosary, Nicole Lesko, John W Yarham, Helene Bruhn, Rolf Wibom, Inger Nennesmo, Richard G Weleber, Emma L Blakely, Robert W Taylor, Robert McFarland
Eur J Hum Genet. 2012 Aug; 20(8): 897–904. Published online 2012 Feb 29. doi: 10.1038/ejhg.2012.44
Correction in:
Eur J Hum Genet. 2012 Aug; 20(8): 910.
PMCID:
PMC3400738
AbstractArticlePDF–630K
10.
Mitochondrial Structure, Function and Dynamics Are Temporally Controlled by c-Myc
J. Anthony Graves, Yudong Wang, Sunder Sims-Lucas, Edward Cherok, Kristi Rothermund, Maria F. Branca, Jennifer Elster, Donna Beer-Stolz, Bennett Van Houten, Jerry Vockley, Edward V. Prochownik
PLoS One. 2012; 7(5): e37699. Published online 2012 May 21. doi: 10.1371/journal.pone.0037699
PMCID:
PMC3357432
AbstractArticlePDF–833K
11.
Partial tandem duplication of mtDNA–tRNAPhe impairs mtDNA translation in late-onset mitochondrial myopathy
Paola Arzuffi, Costanza Lamperti, Erika Fernandez-Vizarra, Paola Tonin, Lucia Morandi, Massimo Zeviani
Neuromuscul Disord. 2012 Jan; 22(1-2): 50–55. doi: 10.1016/j.nmd.2011.07.009
PMCID:
PMC3334271
AbstractArticle
12.
Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons
Fredrik H. Sterky, Alexander F. Hoffman, Dusanka Milenkovic, Betty Bao, Arianna Paganelli, Daniel Edgar, Rolf Wibom, Carl R. Lupica, Lars Olson, Nils-Göran Larsson
Hum Mol Genet. 2012 Mar 1; 21(5): 1078–1089. Published online 2011 Nov 16. doi: 10.1093/hmg/ddr537
PMCID:
PMC3277308
AbstractArticlePDF–445K
13.
Uncoupling the Roles of the SUV3 Helicase in Maintenance of Mitochondrial Genome Stability and RNA Degradation
Xuning Emily Guo, Chi-Fen Chen, Dennis Ding-Hwa Wang, Aram Sandaldjian Modrek, Vy Hoai Phan, Wen-Hwa Lee, Phang-Lang Chen
J Biol Chem. 2011 Nov 4; 286(44): 38783–38794. Published online 2011 Sep 12. doi: 10.1074/jbc.M111.257956
PMCID:
PMC3207411
AbstractArticlePDF–3.4M
14.
Loss of caspase-2 accelerates age-dependent alterations in mitochondrial production of reactive oxygen species
Marisa Lopez-Cruzan, Brian Herman
Biogerontology. 2013; 14(2): 121–130. Published online 2013 Mar 16. doi: 10.1007/s10522-013-9415-x
PMCID:
PMC3657345
AbstractArticlePDF–1.2M
15.
The 90-kDa Heat-shock Protein (Hsp90)-binding Immunophilin FKBP51 Is a Mitochondrial Protein That Translocates to the Nucleus to Protect Cells against Oxidative Stress
Luciana I. Gallo, Mariana Lagadari, Graciela Piwien-Pilipuk, Mario D. Galigniana
J Biol Chem. 2011 Aug 26; 286(34): 30152–30160. Published online 2011 Jul 5. doi: 10.1074/jbc.M111.256610
PMCID:
PMC3191054
AbstractArticlePDF–2.4M
16.
Exome Sequencing Identifies Mitochondrial Alanyl-tRNA Synthetase Mutations in Infantile Mitochondrial Cardiomyopathy
Alexandra Götz, Henna Tyynismaa, Liliya Euro, Pekka Ellonen, Tuulia Hyötyläinen, Tiina Ojala, Riikka H. Hämäläinen, Johanna Tommiska, Taneli Raivio, Matej Oresic, Riitta Karikoski, Outi Tammela, Kalle O.J. Simola, Anders Paetau, Tiina Tyni, Anu Suomalainen
Am J Hum Genet. 2011 May 13; 88(5): 635–642. doi: 10.1016/j.ajhg.2011.04.006
PMCID:
PMC3146718
AbstractArticlePDF–1.0M
17.
A Forward Genetic Screen Identifies Mutants Deficient for Mitochondrial Complex I Assembly in Chlamydomonas reinhardtii
M. Rosario Barbieri, Véronique Larosa, Cécile Nouet, Nitya Subrahmanian, Claire Remacle, Patrice P. Hamel
Genetics. 2011 Jun; 188(2): 349–358. doi: 10.1534/genetics.111.128827
PMCID:
PMC3122308
AbstractArticlePDF–2.6M
18.
Mutant p53 exhibits trivial effects on mitochondrial functions which can be reactivated by ellipticine in lymphoma cells
Fei Wang, Jianfeng Liu, Delira Robbins, Kerri Morris, Amos Sit, Yong-Yu Liu, Yunfeng Zhao
Apoptosis. Author manuscript; available in PMC 2012 Mar 1.
Published in final edited form as: Apoptosis. 2011 Mar; 16(3): 301–310. doi: 10.1007/s10495-010-0559-8
PMCID:
PMC3078632
AbstractArticlePDF–1.4M
19.
Ataxia telangiectasia mutated influences cytochrome c oxidase activity
Akshar Y. Patel, Todd M. McDonald, Larry D. Spears, James Kain Ching, Jonathan S. Fisher
Biochem Biophys Res Commun. Author manuscript; available in PMC 2012 Feb 25.
Published in final edited form as: Biochem Biophys Res Commun. 2011 Feb 25; 405(4): 599–603. Published online 2011 Jan 23. doi: 10.1016/j.bbrc.2011.01.075
PMCID:
PMC3055168
AbstractArticlePDF–485K
20.
Hypoxia-Inducible Factor 1: Regulator of Mitochondrial Metabolism and Mediator of Ischemic Preconditioning
Gregg L. Semenza
Biochim Biophys Acta. Author manuscript; available in PMC 2012 Jul 1.
Published in final edited form as: Biochim Biophys Acta. 2011 Jul; 1813(7): 1263–1268. Published online 2010 Aug 21. doi: 10.1016/j.bbamcr.2010.08.006
PMCID:
PMC3010308
AbstractArticlePDF–370K

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