Publications by authors named "Paule Bénit"

63Publications

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

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

[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

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

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

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

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

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

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

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

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

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

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

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

Life with or without AIF.

Trends Biochem Sci 2010 May 6;35(5):278-87. Epub 2010 Feb 6.

INSERM U848, 94805 Villejuif Cedex, France.

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http://dx.doi.org/10.1016/j.tibs.2009.12.008DOI Listing
May 2010

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

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

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

Three spectrophotometric assays for the measurement of the five respiratory chain complexes in minuscule biological samples.

Clin Chim Acta 2006 Dec 2;374(1-2):81-6. Epub 2006 Jun 2.

Inserm, U676, Paris, F-75019 France and Université Paris 7, Faculté de médecine Denis Diderot, IFR02, Paris, France.

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http://dx.doi.org/10.1016/j.cca.2006.05.034DOI Listing
December 2006

Loss of Aif function causes cell death in the mouse embryo, but the temporal progression of patterning is normal.

Proc Natl Acad Sci U S A 2006 Jun 20;103(26):9918-23. Epub 2006 Jun 20.

Department of Anatomy, University of California-San Francisco, San Francisco, CA 94143, USA.

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http://dx.doi.org/10.1073/pnas.0603950103DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1502554PMC
June 2006

Respiratory chain defects: what do we know for sure about their consequences in vivo?

Biochim Biophys Acta 2004 Dec;1659(2-3):172-7

INSERM U393, Handicaps Génétiques de l'Enfant, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, F-75015, Paris, France.

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http://dx.doi.org/10.1016/j.bbabio.2004.07.002DOI Listing
December 2004

Processing of the 24 kDa subunit mitochondrial import signal is not required for assembly of functional complex I in Yarrowia lipolytica.

Eur J Biochem 2004 Sep;271(17):3588-95

Universität Frankfurt, Fachbereich Medizin, Institut für Biochemie I, Frankfurt am Main, Germany.

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http://dx.doi.org/10.1111/j.0014-2956.2004.04296.xDOI Listing
September 2004

Assay of mitochondrial respiratory chain complex I in human lymphocytes and cultured skin fibroblasts.

Biochem Biophys Res Commun 2003 Jan;301(1):222-4

Unité de Recherches sur les Handicaps Génétiques de l'Enfant (INSERM U393), 2éme Etage, Hôpital Necker-Enfants Malades, 149, rue de Sèvres, 75743 Paris cedex 15, France.

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http://dx.doi.org/10.1016/s0006-291x(02)03016-4DOI Listing
January 2003

Cell complementation using Genebridge 4 human:rodent hybrids for physical mapping of novel mitochondrial respiratory chain deficiency genes.

Hum Mol Genet 2002 Dec;11(26):3273-81

Unité de Recherches sur les Handicaps Génétiques de l'Enfant (INSERM U393), Service Informatique, Service de Cytogénétique, Hôpital Necker-Enfants Malades, Paris, Généthon, Evry, France.

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http://dx.doi.org/10.1093/hmg/11.26.3273DOI Listing
December 2002

Coenzyme Q(10) and idebenone in the therapy of respiratory chain diseases: rationale and comparative benefits.

Mol Genet Metab 2002 Sep-Oct;77(1-2):21-30

Service de Génétique Médicale and Unité de Recherches sur les Handicaps Génétiques de l'Enfant INSERM U-393, Hôpital Necker-Enfants Malades, 149, rue de Sèvres, 75743 15, Paris Cedex, France.

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http://dx.doi.org/10.1016/s1096-7192(02)00145-2DOI Listing
May 2003