PMC Links for GEO Profiles (Select 39438596) - PMC

Select item 56019151.

Therapeutic effects of the mitochondrial ROS-redox modulator KH176 in a mammalian model of Leigh Disease

Ria de Haas, Devashish Das, Alejandro Garanto, Herma G. Renkema, Rick Greupink, Petra van den Broek, Jeanne Pertijs, Rob W. J. Collin, Peter Willems, Julien Beyrath, Arend Heerschap, Frans G. Russel, Jan A. Smeitink

Sci Rep. 2017; 7: 11733. Published online 2017 Sep 15. doi: 10.1038/s41598-017-09417-5

PMCID:: PMC5601915

Abstract Article PDF–6.9M

Select item 53611882.

Conditional deletion of Ndufs4 in dopaminergic neurons promotes Parkinson’s disease-like non-motor symptoms without loss of dopamine neurons

Won-Seok Choi, Hyung-Wook Kim, François Tronche, Richard D. Palmiter, Daniel R. Storm, Zhengui Xia

Sci Rep. 2017; 7: 44989. Published online 2017 Mar 22. doi: 10.1038/srep44989

PMCID:: PMC5361188

Abstract Article PDF–1.7M

Select item 47528213.

Assessing the Mitochondrial Membrane Potential in Cells and In Vivo using Targeted Click Chemistry and Mass Spectrometry

Angela Logan, Victoria R. Pell, Karl J. Shaffer, Cameron Evans, Nathan J. Stanley, Ellen L. Robb, Tracy A. Prime, Edward T. Chouchani, Helena M. Cochemé, Ian M. Fearnley, Sara Vidoni, Andrew M. James, Carolyn M. Porteous, Linda Partridge, Thomas Krieg, Robin A.J. Smith, Michael P. Murphy

Cell Metab. 2016 Feb 9; 23(2): 379–385. doi: 10.1016/j.cmet.2015.11.014

PMCID:: PMC4752821

Abstract Article

Select item 47326144.

Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse Brain

Ernst-Bernhard Kayser, Margaret M. Sedensky, Philip G. Morgan

PLoS One. 2016; 11(1): e0148219. Published online 2016 Jan 29. doi: 10.1371/journal.pone.0148219

PMCID:: PMC4732614

Abstract Article PDF–3.1M

Select item 46380455.

Interacting Network of the Gap Junction (GJ) Protein Connexin43 (Cx43) is Modulated by Ischemia and Reperfusion in the Heart

Tania Martins-Marques, Sandra Isabel Anjo, Paulo Pereira, Bruno Manadas, Henrique Girão

Mol Cell Proteomics. 2015 Nov; 14(11): 3040–3055. Published online 2015 Aug 27. doi: 10.1074/mcp.M115.052894

PMCID:: PMC4638045

Abstract Article PDF–2.5M

Select item 44062966.

Mitochondrial complex I deficiency leads to inflammation and retinal ganglion cell death in the Ndufs4 mouse

Alfred K. Yu, Lanying Song, Karl D. Murray, Deborah van der List, Chao Sun, Yan Shen, Zhengui Xia, Gino A. Cortopassi

Hum Mol Genet. 2015 May 15; 24(10): 2848–2860. Published online 2015 Feb 4. doi: 10.1093/hmg/ddv045

PMCID:: PMC4406296

Abstract Article PDF–1.3M

Select item 39290837.

Partial complex I deficiency due to the CNS conditional ablation of Ndufa5 results in a mild chronic encephalopathy but no increase in oxidative damage

Susana Peralta, Alessandra Torraco, Tina Wenz, Sofia Garcia, Francisca Diaz, Carlos T. Moraes

Hum Mol Genet. 2014 Mar 15; 23(6): 1399–1412. Published online 2013 Oct 23. doi: 10.1093/hmg/ddt526

PMCID:: PMC3929083

Abstract Article PDF–1.4M

Select item 33878178.

Fatal breathing dysfunction in a mouse model of Leigh syndrome

Albert Quintana, Sebastien Zanella, Henner Koch, Shane E. Kruse, Donghoon Lee, Jan M. Ramirez, Richard D. Palmiter

J Clin Invest. 2012 Jul 2; 122(7): 2359–2368. Published online 2012 Jun 1. doi: 10.1172/JCI62923

PMCID:: PMC3387817

Abstract Article PDF–2.1M

Select item 33702489.

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

Dillon W. Leong, Jasper C. Komen, Chelsee A. Hewitt, Estelle Arnaud, Matthew McKenzie, Belinda Phipson, Melanie Bahlo, Adrienne Laskowski, Sarah A. Kinkel, Gayle M. Davey, William R. Heath, Anne K. Voss, René P. Zahedi, James J. Pitt, Roman Chrast, Albert Sickmann, Michael T. Ryan, Gordon K. Smyth, David R. Thorburn, Hamish S. Scott

J Biol Chem. 2012 Jun 8; 287(24): 20652–20663. Published online 2012 Apr 25. doi: 10.1074/jbc.M111.327601

PMCID:: PMC3370248

Abstract Article PDF–2.6M

Select item 319069410.

d-Amino Acid Oxidase Activity Is Inhibited by an Interaction with Bassoon Protein at the Presynaptic Active Zone

Michael Popiolek, John F. Ross, Erik Charych, Pranab Chanda, Eckart D. Gundelfinger, Stephen J. Moss, Nicholas J. Brandon, Mark H. Pausch

J Biol Chem. 2011 Aug 19; 286(33): 28867–28875. Published online 2011 Jun 23. doi: 10.1074/jbc.M111.262063

PMCID:: PMC3190694

Abstract Article PDF–1.0M

Select item 317805911.

Phylogenetic-based propagation of functional annotations within the Gene Ontology consortium

Pascale Gaudet, Michael S. Livstone, Suzanna E. Lewis, Paul D. Thomas

Brief Bioinform. 2011 Sep; 12(5): 449–462. Published online 2011 Aug 27. doi: 10.1093/bib/bbr042

PMCID:: PMC3178059

Abstract Article PDF–1.0M

Select item 305182012.

Loss of mitochondrial complex I activity potentiates dopamine neuron death induced by microtubule dysfunction in a Parkinson’s disease model

Won-Seok Choi, Richard D. Palmiter, Zhengui Xia

J Cell Biol. 2011 Mar 7; 192(5): 873–882. doi: 10.1083/jcb.201009132

PMCID:: PMC3051820

Abstract Article PDF–1.7M

Select item 289071713.

Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome

Albert Quintana, Shane E. Kruse, Raj P. Kapur, Elisenda Sanz, Richard D. Palmiter

Proc Natl Acad Sci U S A. 2010 Jun 15; 107(24): 10996–11001. Published online 2010 Jun 1. doi: 10.1073/pnas.1006214107

PMCID:: PMC2890717

Abstract Article PDF–1.1M

Select item 277884414.

A mitochondrial protein compendium elucidates complex I disease biology

David J. Pagliarini, Sarah E. Calvo, Betty Chang, Sunil A. Sheth, Scott B. Vafai, Shao-En Ong, Geoffrey A. Walford, Canny Sugiana, Avihu Boneh, William K. Chen, David E. Hill, Marc Vidal, James G. Evans, David R. Thorburn, Steven A. Carr, Vamsi K. Mootha

Cell. Author manuscript; available in PMC 2009 Nov 18.

Published in final edited form as: Cell. 2008 Jul 11; 134(1): 112–123. doi: 10.1016/j.cell.2008.06.016