Pierre Rustin

Pierre Rustin

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Alternative respiratory chain enzymes: Therapeutic potential and possible pitfalls.

Biochim Biophys Acta Mol Basis Dis 2019 04 17;1865(4):854-866. Epub 2018 Oct 17.

Faculty of Medicine and Life Sciences, BioMediTech Institute and Tampere University Hospital, FI-33014, University of Tampere, Finland; Institute of Biotechnology, FI-00014, University of Helsinki, Finland. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S09254439183038
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http://dx.doi.org/10.1016/j.bbadis.2018.10.012DOI Listing
April 2019

Distinctive Krebs cycle remodeling in iPSC-derived neural and mesenchymal stem cells.

Biochem Biophys Res Commun 2019 04 27;511(3):658-664. Epub 2019 Feb 27.

INSERM UMR 1141, Hôpital Robert Debré, 48 Boulevard Sérurier, 75019, Paris, France; Université Paris Diderot, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.bbrc.2019.02.033DOI Listing
April 2019

CHCHD2 accumulates in distressed mitochondria and facilitates oligomerization of CHCHD10.

Hum Mol Genet 2019 01;28(2):349

Inherited Movement Disorders Unit, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, USA.

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http://dx.doi.org/10.1093/hmg/ddy340DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6322067PMC
January 2019

CHCHD2 accumulates in distressed mitochondria and facilitates oligomerization of CHCHD10.

Hum Mol Genet 2018 11;27(22):3881-3900

Inherited Movement Disorders Unit, Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD USA.

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http://dx.doi.org/10.1093/hmg/ddy270DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6216204PMC
November 2018

mutations: A novel phenotype associating severe optic atrophy and spastic paraplegia.

Neurol Genet 2018 Apr 20;4(2):e225. Epub 2018 Mar 20.

Department of Neurology (C.M., B.C.), Gui de Chauliac Montpellier University Hospital; EA7402 Institut Universitaire de Recherche Clinique (C.M., L.L., M.K., C.G.), and Laboratoire de Génétique Moléculaire, University Hospital; Maladies Sensorielles Génétiques (C.H., M.Q., C.D., E.S.), CHRU; INSERM U1051 (C.H., M.Q., C.D., E.S.), Institute for Neurosciences of Montpellier; Université Montpellier (C.H., M.Q., C.D., E.S.), France; INSERM UMR 1141 (D.C., P.R.), PROTECT, INSERM, Université Paris Diderot, Sorbonne Paris Cité, France.

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http://dx.doi.org/10.1212/NXG.0000000000000225DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5860906PMC
April 2018

Paradoxical Inhibition of Glycolysis by Pioglitazone Opposes the Mitochondriopathy Caused by AIF Deficiency.

EBioMedicine 2017 Mar 16;17:75-87. Epub 2017 Feb 16.

INSERM UMR 1141, PROTECT, INSERM, Université Paris Diderot, Sorbonne Paris Cité, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.ebiom.2017.02.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5360583PMC
March 2017

Genetic and biochemical intricacy shapes mitochondrial cytopathies.

Neurobiol Dis 2016 08 12;92(Pt A):55-63. Epub 2015 Feb 12.

INSERM UMR 1141, Hôpital Robert Debré, Paris, France; Université Paris 7, Faculté de Médecine Denis Diderot, Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.nbd.2015.02.003DOI Listing
August 2016

Translocator Protein-Mediated Stabilization of Mitochondrial Architecture during Inflammation Stress in Colonic Cells.

PLoS One 2016 7;11(4):e0152919. Epub 2016 Apr 7.

Sorbonne Universités - Université Pierre et Marie Curie Université de Paris VI, École Normale Supérieure - PSL Research University, Département de Chimie, CNRS UMR 7203 LBM, 4 Place Jussieu, F-75005, Paris, France.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0152919PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4824355PMC
August 2016

Expression of the alternative oxidase mitigates beta-amyloid production and toxicity in model systems.

Free Radic Biol Med 2016 07 14;96:57-66. Epub 2016 Apr 14.

INSERM UMR 1141 and Université Paris 7, Faculté de Médecine Denis Diderot, Hôpital Robert Debré, 48, Boulevard Sérurier, 75019 Paris, France.

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http://dx.doi.org/10.1016/j.freeradbiomed.2016.04.006DOI Listing
July 2016

Idebenone in Friedreich ataxia and Leber's hereditary optic neuropathy: close mechanisms, similar therapy?

Brain 2016 07 19;139(Pt 7):e39. Epub 2016 Apr 19.

1 Institut National de la Santé et de la Recherche Médicale Unité Mixte de Recherche 1141, Hôpital Robert Debré, 48 Boulevard Sérurier, 75019 Paris, France 2 Faculté de Médecine Denis Diderot, Université Paris Diderot - Paris 7, Site Robert Debré, 48 Boulevard Sérurier, 75019 Paris, France

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http://dx.doi.org/10.1093/brain/aww085DOI Listing
July 2016

[Heart failure from mitochondrial origin: a matter of diet?].

Med Sci (Paris) 2016 May 25;32(5):458-60. Epub 2016 May 25.

Inserm UMR1141, Hôpital Robert Debré, 48, boulevard Sérurier, 75019 Paris, France.

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http://dx.doi.org/10.1051/medsci/20163205011DOI Listing
May 2016

DAPIT Over-Expression Modulates Glucose Metabolism and Cell Behaviour in HEK293T Cells.

PLoS One 2015 10;10(7):e0131990. Epub 2015 Jul 10.

Department of Biology of Physical Activity, University of Jyväskylä, Jyväskylä, Finland.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0131990PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4498893PMC
April 2016

In Vivo Detection of Succinate by Magnetic Resonance Spectroscopy as a Hallmark of SDHx Mutations in Paraganglioma.

Clin Cancer Res 2016 Mar 21;22(5):1120-9. Epub 2015 Oct 21.

INSERM, UMR970, Paris Cardiovascular Research Center, Paris, France. Université Paris Descartes, Sorbonne Paris Cité, Faculté de Médecine, Paris, France. Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Radiologie, Paris, France.

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http://dx.doi.org/10.1158/1078-0432.CCR-15-1576DOI Listing
March 2016

Mitochondrial cytochrome c oxidase deficiency.

Clin Sci (Lond) 2016 Mar;130(6):393-407

Institut National de la Santé et de la Recherche Médicale Unité Mixte de Recherche 1141, Hôpital Robert Debré, 48 Boulevard Sérurier, 75019 Paris, France Faculté de Médecine Denis Diderot, Université Paris Diderot-Paris 7, Site Robert Debré, 48 Boulevard Sérurier, 75019 Paris, France

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http://dx.doi.org/10.1042/CS20150707DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4948581PMC
March 2016

From Nf1 to Sdhb knockout: Successes and failures in the quest for animal models of pheochromocytoma.

Mol Cell Endocrinol 2016 Feb 27;421:40-8. Epub 2015 Jun 27.

INSERM, UMR970, Paris-Cardiovascular Research Center, F-75015 Paris, France; Université Paris Descartes, Sorbonne Paris Cité, Faculté de Médecine, F-75006 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.mce.2015.06.027DOI Listing
February 2016

Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice.

Science 2015 Dec;350(6265):aad0116

Institute for Genetics, University of Cologne, 50674 Cologne, Germany. Max-Planck-Institute for Biology of Aging, Cologne, Germany. Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne, Cologne, Germany. Center for Molecular Medicine (CMMC), University of Cologne, Cologne, Germany.

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http://dx.doi.org/10.1126/science.aad0116DOI Listing
December 2015

ANT2-defective fibroblasts exhibit normal mitochondrial bioenergetics.

Mol Genet Metab Rep 2015 Jun;3:43-46

Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA ; Inserm U1141, Université Paris-Diderot, Sorbonne Paris Cité, Paris, France ; Reference Center for Inborn Errors of Metabolism, Robert Debré University Hospital, APHP, Paris, France.

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http://dx.doi.org/10.1016/j.ymgmr.2015.03.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4435574PMC
June 2015

Interaction between AIF and CHCHD4 Regulates Respiratory Chain Biogenesis.

Mol Cell 2015 Jun 21;58(6):1001-14. Epub 2015 May 21.

Equipe 11 labellisée par la Ligue Nationale contre le Cancer, Centre de Recherche des Cordeliers, 75006 Paris, France; INSERM, UMRS 1138, 75006 Paris, France; Gustave Roussy Comprehensive Cancer Center, 94805 Villejuif, France; Université Paris Descartes/Paris V, Sorbonne Paris Cité, 75006 Paris, France; Université Pierre et Marie Curie, 75006 Paris, France. Electronic address:

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http://dx.doi.org/10.1016/j.molcel.2015.04.020DOI Listing
June 2015

Unsuspected task for an old team: succinate, fumarate and other Krebs cycle acids in metabolic remodeling.

Biochim Biophys Acta 2014 Aug 31;1837(8):1330-7. Epub 2014 Mar 31.

Hôpital Robert Debré, INSERM, UMR1141, Paris 75019, France; U.F.R. de Médecine Université Paris Diderot, Paris 75019, France. Electronic address:

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http://dx.doi.org/10.1016/j.bbabio.2014.03.013DOI Listing
August 2014

Friedreich's ataxia, frataxin, PIP5K1B: echo of a distant fracas.

Oxid Med Cell Longev 2013 30;2013:725635. Epub 2013 Sep 30.

INSERM UMR 676, Bâtiment Ecran, Hôpital Robert Debré, 48 boulevard Sérurier, 75019 Paris, France ; Université Paris 7, Faculté de Médecine Denis Diderot, Site Robert Debré, 48 boulevard Sérurier, 75019 Paris, France.

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http://dx.doi.org/10.1155/2013/725635DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3806116PMC
May 2014

Effect of Lon protease knockdown on mitochondrial function in HeLa cells.

Biochimie 2014 May 17;100:38-47. Epub 2013 Dec 17.

UR4 - Vieillissement, Stress, Inflammation, Sorbonne Universités, UPMC Univ Paris 06, Université Pierre et Marie Curie, 4 Place Jussieu, 75252 Paris Cedex 05, France.

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http://dx.doi.org/10.1016/j.biochi.2013.12.005DOI Listing
May 2014

Supernumerary subunits NDUFA3, NDUFA5 and NDUFA12 are required for the formation of the extramembrane arm of human mitochondrial complex I.

FEBS Lett 2014 May 6;588(9):1832-8. Epub 2014 Apr 6.

INSERM UMR 1141, Bâtiment Ecran, Hôpital Robert Debré, 48 Boulevard Serurier, 75019 Paris, France.

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http://dx.doi.org/10.1016/j.febslet.2014.03.046DOI Listing
May 2014

Expression of alternative oxidase in Drosophila ameliorates diverse phenotypes due to cytochrome oxidase deficiency.

Hum Mol Genet 2014 Apr 29;23(8):2078-93. Epub 2013 Nov 29.

Institute of Biomedical Technology and Tampere University Hospital, University of Tampere, FI-33014 Tampere, Finland.

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http://dx.doi.org/10.1093/hmg/ddt601DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3959817PMC
April 2014

Cis-silencing of PIP5K1B evidenced in Friedreich's ataxia patient cells results in cytoskeleton anomalies.

Hum Mol Genet 2013 Jul 2;22(14):2894-904. Epub 2013 Apr 2.

Hôpital Robert Debré, INSERM UMR 676 Faculté de Médecine Denis Diderot, Université Paris 7, 75019 Paris, France.

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http://dx.doi.org/10.1093/hmg/ddt144DOI Listing
July 2013

Disconnecting mitochondrial content from respiratory chain capacity in PGC-1-deficient skeletal muscle.

Cell Rep 2013 May 23;3(5):1449-56. Epub 2013 May 23.

Cardiovascular Institute, Department of Pathology, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA 02215, USA.

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http://dx.doi.org/10.1016/j.celrep.2013.04.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3688451PMC
May 2013

PGC-1α is dispensable for exercise-induced mitochondrial biogenesis in skeletal muscle.

PLoS One 2012 24;7(7):e41817. Epub 2012 Jul 24.

Cardiovascular Institute, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, United States of America.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0041817PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3404101PMC
April 2013

Glucose modulates respiratory complex I activity in response to acute mitochondrial dysfunction.

J Biol Chem 2012 Nov 24;287(46):38729-40. Epub 2012 Sep 24.

Institute of Biomedical Technology and Centre for Laboratory Medicine, Tampere University Hospital, University of Tampere, 33014 Tampere, Finland.

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http://dx.doi.org/10.1074/jbc.M112.386060DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3493916PMC
November 2012

Therapies in inborn errors of oxidative metabolism.

Trends Endocrinol Metab 2012 Sep 25;23(9):488-95. Epub 2012 May 25.

Institut National de la Santé et de la Recherche Médicale Unité 676, Hôpital Robert Debré, F-75019 Paris, France.

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http://dx.doi.org/10.1016/j.tem.2012.04.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4135311PMC
September 2012

HIF1α and pancreatic β-cell development.

FASEB J 2012 Jul 16;26(7):2734-42. Epub 2012 Mar 16.

Institut National de Santé et de Recherche Médicale (INSERM) U845, Research Center Growth and Signalling, Université Paris Descartes, Sorbonne Paris Cité, Faculté de Médecine, Paris, France.

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http://dx.doi.org/10.1096/fj.11-199224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4046099PMC
July 2012

Changing the diet to make more mitochondria and protect the heart.

Circ Res 2012 Apr;110(8):1047-8

Inserm U676, Hôpital Robert Debré, Paris, France.

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http://dx.doi.org/10.1161/RES.0b013e318255408aDOI Listing
April 2012

Succinate dehydrogenase (SDH) D subunit (SDHD) inactivation in a growth-hormone-producing pituitary tumor: a new association for SDH?

J Clin Endocrinol Metab 2012 Mar 14;97(3):E357-66. Epub 2011 Dec 14.

Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development (NICHD), National Institutes of Health (NIH), Building 10, CRC, Room 1-3330, 10 Center Drive, MSC1103, Bethesda, Maryland 20892, USA.

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http://dx.doi.org/10.1210/jc.2011-1179DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3319210PMC
March 2012

Mitochondrial complex I deficiency of nuclear origin II. Non-structural genes.

Mol Genet Metab 2012 Feb 20;105(2):173-9. Epub 2011 Oct 20.

Laboratoire de Biochimie, APHP Hôpital de Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin Bicêtre cedex, France.

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http://dx.doi.org/10.1016/j.ymgme.2011.10.001DOI Listing
February 2012

Mitochondrial complex I deficiency of nuclear origin I. Structural genes.

Mol Genet Metab 2012 Feb 18;105(2):163-72. Epub 2011 Nov 18.

Laboratoire de Biochimie, APHP Hôpital de Bicêtre, 78 rue du Général Leclerc, 94275 Le Kremlin Bicêtre cedex, France.

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http://dx.doi.org/10.1016/j.ymgme.2011.11.188DOI Listing
February 2012

Oxidative stress induces mitochondrial fragmentation in frataxin-deficient cells.

Biochem Biophys Res Commun 2012 Feb 17;418(2):336-41. Epub 2012 Jan 17.

Mitochondria, Metals and Oxidative Stress Laboratory, Institut Jacques Monod, CNRS-Université Paris-Diderot, Sorbonne Paris Cité, 15 rue Hélène Brion, 75205 Paris cedex 13, France.

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http://dx.doi.org/10.1016/j.bbrc.2012.01.022DOI Listing
February 2012

Mutations in the dimer interface of dihydrolipoamide dehydrogenase promote site-specific oxidative damages in yeast and human cells.

J Biol Chem 2011 Nov 19;286(46):40232-45. Epub 2011 Sep 19.

Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester Minnesota 55905, USA.

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http://www.jbc.org/lookup/doi/10.1074/jbc.M111.274415
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http://dx.doi.org/10.1074/jbc.M111.274415DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3220568PMC
November 2011

Friedreich's ataxia: the vicious circle hypothesis revisited.

BMC Med 2011 Oct 11;9:112. Epub 2011 Oct 11.

Inserm, U676, Physiopathology and Therapy of Mitochondrial Diseases Laboratory, CHU - Hôpital Robert Debré, 48, boulevard Sérurier, F-75019 Paris, France.

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http://dx.doi.org/10.1186/1741-7015-9-112DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3198887PMC
October 2011

Developmental aspects of respiratory chain from fetus to infancy.

Semin Fetal Neonatal Med 2011 Aug 2;16(4):175-80. Epub 2011 Jun 2.

INSERM U676, Hôpital Robert Debré, 75019 Paris, France.

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http://dx.doi.org/10.1016/j.siny.2011.05.005DOI Listing
August 2011

Mitochondrial response to controlled nutrition in health and disease.

Nutr Rev 2011 Feb 14;69(2):65-75. Epub 2011 Jan 14.

Centre de référence Maladies Métaboliques, Hôpital Robert Debré, APHP, Université Paris 7, Faculté de médecine Denis Diderot, IFR02, INSERM, U676, Paris, France.

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http://dx.doi.org/10.1111/j.1753-4887.2010.00363.xDOI Listing
February 2011

Stabilization of hypoxia-inducible factor-1alpha protein in hypoxia occurs independently of mitochondrial reactive oxygen species production.

J Biol Chem 2010 Oct 30;285(41):31277-84. Epub 2010 Jul 30.

Department of Biochemistry, Yong Loo Lin School of Medicine, National University of Singapore, 117597 Singapore.

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http://dx.doi.org/10.1074/jbc.M110.158485DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2951202PMC
October 2010

SDHA is a tumor suppressor gene causing paraganglioma.

Hum Mol Genet 2010 Aug 18;19(15):3011-20. Epub 2010 May 18.

Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Génétique, 20-40 rue Leblanc, F-75015 Paris, France.

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http://dx.doi.org/10.1093/hmg/ddq206DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2901140PMC
August 2010

A critical approach to the therapy of mitochondrial respiratory chain and oxidative phosphorylation diseases.

Biochim Biophys Acta 2009 Dec 5;1792(12):1159-67. Epub 2008 Nov 5.

Department of Neurology, Columbia University Medical Center, 313 Russ Berrie Medical Science Pavilion, 1150 St. Nicholas Avenue, New York, NY 10032, USA.

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http://linkinghub.elsevier.com/retrieve/pii/S092544390800200
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http://dx.doi.org/10.1016/j.bbadis.2008.10.015DOI Listing
December 2009

The alternative oxidase, a tool for compensating cytochrome c oxidase deficiency in human cells.

Physiol Plant 2009 Dec 25;137(4):427-34. Epub 2009 Apr 25.

Inserm, U676 and Université Paris 7, Faculté de Médecine Denis Diderot, Paris, France.

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http://dx.doi.org/10.1111/j.1399-3054.2009.01248.xDOI Listing
December 2009

Respiratory chain alternative enzymes as tools to better understand and counteract respiratory chain deficiencies in human cells and animals.

Physiol Plant 2009 Dec 6;137(4):362-70. Epub 2009 May 6.

INSERM U676 and Université Paris 7, Faculté de Médecine Denis Diderot, Paris, France.

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http://doi.wiley.com/10.1111/j.1399-3054.2009.01249.x
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http://dx.doi.org/10.1111/j.1399-3054.2009.01249.xDOI Listing
December 2009

Limitations in a frataxin knockdown cell model for Friedreich ataxia in a high-throughput drug screen.

BMC Neurol 2009 Aug 24;9:46. Epub 2009 Aug 24.

IGBMC (Institut de Génétique et de Biologie Moléculaire et Cellulaire), CNRS/INSERM/Université Louis Pasteur, Illkirch cedex, France.

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http://bmcneurol.biomedcentral.com/articles/10.1186/1471-237
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http://dx.doi.org/10.1186/1471-2377-9-46DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2744904PMC
August 2009

Respiratory-chain diseases related to complex III deficiency.

Biochim Biophys Acta 2009 Jan 13;1793(1):181-5. Epub 2008 Jun 13.

Inserm, U676, Hôpital Robert Debré, Paris, F-75019, France.

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http://dx.doi.org/10.1016/j.bbamcr.2008.06.004DOI Listing
January 2009

[Mitochondrial disorders].

Bull Acad Natl Med 2009 Jan;193(1):19-41; discussion 41-3

Pédiatrie, Hôpital Necker-Enfants malades, INSERM U393, 149 rue de Sèvres, 75743 Paris.

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January 2009

The optimized allotopic expression of ND1 or ND4 genes restores respiratory chain complex I activity in fibroblasts harboring mutations in these genes.

Biochim Biophys Acta 2008 Oct 6;1783(10):1707-17. Epub 2008 May 6.

Institut de la Vision, Université Pierre et Marie Curie-Paris, INSERM UMR-S 592, 17 rue Moreau, Paris, F-75012 France.

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http://dx.doi.org/10.1016/j.bbamcr.2008.04.018DOI Listing
October 2008

Decylubiquinol impedes mitochondrial respiratory chain complex I activity.

Mol Cell Biochem 2008 Jul 15;314(1-2):45-50. Epub 2008 Apr 15.

Inserm, U676, Hôpital Robert Debré, Bât Ecran, Paris, France.

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http://dx.doi.org/10.1007/s11010-008-9763-zDOI Listing
July 2008

Deferiprone targets aconitase: implication for Friedreich's ataxia treatment.

BMC Neurol 2008 Jun 16;8:20. Epub 2008 Jun 16.

Inserm, U676, Hôpital Robert Debré, Paris, F-75019 France and Université Paris 7, Faculté de Médecine Denis Diderot, IFR02, Paris, France.

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http://dx.doi.org/10.1186/1471-2377-8-20DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2440763PMC
June 2008

The mtDNA NARP mutation activates the actin-Nrf2 signaling of antioxidant defenses.

Biochem Biophys Res Commun 2008 Apr 6;368(3):620-4. Epub 2008 Feb 6.

Inserm, U676, Hôpital Robert Debré, 48 Boulevard Serurier, Batiment Ecran, Paris F-75019, France.

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http://dx.doi.org/10.1016/j.bbrc.2008.01.125DOI Listing
April 2008

A cancer-predisposing "hot spot" mutation of the fumarase gene creates a dominant negative protein.

Int J Cancer 2008 Feb;122(4):947-51

Laboratory of Cancer Genetics, Institute for Cancer Research and Treatment (IRCC), University of Turin Medical School, SP142, KM 395, 10060 Candiolo, Turin, Italy.

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http://dx.doi.org/10.1002/ijc.23209DOI Listing
February 2008

Allotopic mRNA localization to the mitochondrial surface rescues respiratory chain defects in fibroblasts harboring mitochondrial DNA mutations affecting complex I or v subunits.

Rejuvenation Res 2007 Jun;10(2):127-44

Laboratoire de Physiopathologie Cellulaire et Moléculaire de la Rétine, INSERM U592, Université Pierre et Marie Curie (UPMC-Paris6), Hôpital St. Antoine-Bât. Kourilsky, 184 rue du Faubourg Saint-Antoine, 75571 Paris Cedex 12, France.

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http://dx.doi.org/10.1089/rej.2006.0526DOI Listing
June 2007