Publications by authors named "Malgorzata Rak"

36Publications

Deregulating mitochondrial metabolite and ion transport has beneficial effects in yeast and human cellular models for NARP syndrome.

Hum Mol Genet 2019 11;28(22):3792-3804

Institut de Biochimie et Génétique Cellulaires, Université Victor Segalen, 1 Rue Camille Saint-Saëns, CEDEX 33077 Bordeaux, France.

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http://dx.doi.org/10.1093/hmg/ddz160DOI Listing
November 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

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

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

Regulation of mitochondrial translation of the ATP8/ATP6 mRNA by Smt1p.

Mol Biol Cell 2016 Mar 28;27(6):919-29. Epub 2016 Jan 28.

Department of Biological Sciences, Columbia University, New York, NY 10027

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http://dx.doi.org/10.1091/mbc.E15-09-0642DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4791136PMC
March 2016

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

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

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

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

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

Characterization of Gtf1p, the connector subunit of yeast mitochondrial tRNA-dependent amidotransferase.

J Biol Chem 2011 Sep 28;286(38):32937-47. Epub 2011 Jul 28.

Department of Microbiology, University of São Paulo, 05508-900 São Paulo, Brazil.

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http://dx.doi.org/10.1074/jbc.M111.265371DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3190888PMC
September 2011

Turnover of ATP synthase subunits in F1-depleted HeLa and yeast cells.

FEBS Lett 2011 Aug 23;585(16):2582-6. Epub 2011 Jul 23.

Department of Biological Sciences, Columbia University, New York, NY 10027, USA.

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http://dx.doi.org/10.1016/j.febslet.2011.07.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3157936PMC
August 2011

Modular assembly of yeast mitochondrial ATP synthase.

EMBO J 2011 Mar 25;30(5):920-30. Epub 2011 Jan 25.

Department of Biological Sciences, Columbia University, New York, NY, USA.

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http://emboj.embopress.org/cgi/doi/10.1038/emboj.2010.364
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http://dx.doi.org/10.1038/emboj.2010.364DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3049208PMC
March 2011

F1-dependent translation of mitochondrially encoded Atp6p and Atp8p subunits of yeast ATP synthase.

Proc Natl Acad Sci U S A 2009 Nov 19;106(44):18509-14. Epub 2009 Oct 19.

Department of Biological Sciences, Columbia University, New York, NY 10027, USA.

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http://dx.doi.org/10.1073/pnas.0910351106DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2774022PMC
November 2009

Biochemical consequences in yeast of the human mitochondrial DNA 8993T>C mutation in the ATPase6 gene found in NARP/MILS patients.

Biochim Biophys Acta 2009 May 6;1793(5):817-24. Epub 2009 Mar 6.

Institut de Biochimie et Génétique Cellulaires, CNRS/Université Victor Segalen Bordeaux2, France.

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http://dx.doi.org/10.1016/j.bbamcr.2009.02.011DOI Listing
May 2009

Assembly of F0 in Saccharomyces cerevisiae.

Biochim Biophys Acta 2009 Jan 11;1793(1):108-16. Epub 2008 Jul 11.

Department of Biological Sciences, Columbia University New York, NY 10027, USA.

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http://dx.doi.org/10.1016/j.bbamcr.2008.07.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2633655PMC
January 2009

Mitochondrial ATP synthase disorders: molecular mechanisms and the quest for curative therapeutic approaches.

Biochim Biophys Acta 2009 Jan 20;1793(1):186-99. Epub 2008 Jun 20.

Institut de Biochimie et Génétique Cellulaires, CNRS-Université Bordeaux2, Bordeaux 33077, France.

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https://linkinghub.elsevier.com/retrieve/pii/S01674889080023
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http://dx.doi.org/10.1016/j.bbamcr.2008.06.012DOI Listing
January 2009

A yeast model of the neurogenic ataxia retinitis pigmentosa (NARP) T8993G mutation in the mitochondrial ATP synthase-6 gene.

J Biol Chem 2007 Nov 12;282(47):34039-47. Epub 2007 Sep 12.

Institut de Biochimie et Génétique Cellulaires CNRS, Université Victor Segalen Bordeaux 2, 1 Rue Camille Saint-Saëns, Bordeaux Cedex, France.

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http://dx.doi.org/10.1074/jbc.M703053200DOI Listing
November 2007

Yeast cells lacking the mitochondrial gene encoding the ATP synthase subunit 6 exhibit a selective loss of complex IV and unusual mitochondrial morphology.

J Biol Chem 2007 Apr 29;282(15):10853-64. Epub 2007 Jan 29.

Institut de Biochimie et Génétique Cellulaires, Université Victor Segalen, 1 Rue Camille Saint-Saëns, 33077 Bordeaux cedex, France.

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http://dx.doi.org/10.1074/jbc.M608692200DOI Listing
April 2007

A "petite obligate" mutant of Saccharomyces cerevisiae: functional mtDNA is lethal in cells lacking the delta subunit of mitochondrial F1-ATPase.

J Biol Chem 2006 Jun 11;281(24):16305-13. Epub 2006 Apr 11.

Institut de Biochimie et Génétique Cellulaires, Université Victor Segalen, 1 Rue Camille Saint-Saëns, Bordeaux 33077 cedex, France.

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http://www.jbc.org/lookup/doi/10.1074/jbc.M513805200
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http://dx.doi.org/10.1074/jbc.M513805200DOI Listing
June 2006

Increasing mitochondrial substrate-level phosphorylation can rescue respiratory growth of an ATP synthase-deficient yeast.

J Biol Chem 2005 Sep 23;280(35):30751-9. Epub 2005 Jun 23.

Institut de Biochimie et Génétique Cellulaires CNRS/Université Victor Ségalen Bordeaux2, 1 rue Camille St Saëns, 33077 Bordeaux Cedex, France.

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http://dx.doi.org/10.1074/jbc.M501831200DOI Listing
September 2005