Johannes N Spelbrink

Johannes N Spelbrink

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Johannes N Spelbrink

Johannes N Spelbrink

Publications by authors named "Johannes N Spelbrink"

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A Combined Mass Spectrometry and Data Integration Approach to Predict the Mitochondrial Poly(A) RNA Interacting Proteome.

Front Cell Dev Biol 2019 15;7:283. Epub 2019 Nov 15.

Department of Pediatrics, Radboud Center for Mitochondrial Medicine, Radboud University Medical Centre, Nijmegen, Netherlands.

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http://dx.doi.org/10.3389/fcell.2019.00283DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6873792PMC
November 2019

Mitochondrial RNA granules are critically dependent on mtDNA replication factors Twinkle and mtSSB.

Nucleic Acids Res 2019 04;47(7):3680-3698

Radboud Center for Mitochondrial Medicine, Department of Paediatrics, Radboudumc, Nijmegen, The Netherlands.

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http://dx.doi.org/10.1093/nar/gkz047DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6468249PMC
April 2019

Transcript availability dictates the balance between strand-asynchronous and strand-coupled mitochondrial DNA replication.

Nucleic Acids Res 2018 11;46(20):10771-10781

Department of Clinical Movement Neurosciences, Institute of Neurology, Royal Free Campus, University College London, London NW3 2PF, UK.

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http://dx.doi.org/10.1093/nar/gky852DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6237803PMC
November 2018

The mitochondrial outer-membrane location of the EXD2 exonuclease contradicts its direct role in nuclear DNA repair.

Sci Rep 2018 03 29;8(1):5368. Epub 2018 Mar 29.

Radboud Center for Mitochondrial Medicine, Department of Paediatrics, Radboudumc, Nijmegen, The Netherlands.

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http://dx.doi.org/10.1038/s41598-018-23690-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5876329PMC
March 2018

Whole cell formaldehyde cross-linking simplifies purification of mitochondrial nucleoids and associated proteins involved in mitochondrial gene expression.

PLoS One 2015 19;10(2):e0116726. Epub 2015 Feb 19.

Mitochondrial DNA Maintenance Group, BioMediTech, FI-33014 University of Tampere, Tampere, Finland; Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders, Radboud University Medical Centre, Geert Grooteplein 10, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0116726PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4335056PMC
January 2016

The hexameric structure of the human mitochondrial replicative helicase Twinkle.

Nucleic Acids Res 2015 Apr 30;43(8):4284-95. Epub 2015 Mar 30.

Structural MitoLab; Department of Structural Biology, Molecular Biology Institute Barcelona (IBMB-CSIC), Barcelona, E-08028, Spain

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http://dx.doi.org/10.1093/nar/gkv189DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4417153PMC
April 2015

What cost mitochondria? The maintenance of functional mitochondrial DNA within and across generations.

Philos Trans R Soc Lond B Biol Sci 2014 Jul;369(1646):20130438

Behaviour and Genetics of Social Insects Lab, The University of Sydney, Sydney NSW 2006, Australia.

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http://rstb.royalsocietypublishing.org/content/royptb/369/16
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http://rstb.royalsocietypublishing.org/cgi/doi/10.1098/rstb.
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http://dx.doi.org/10.1098/rstb.2013.0438DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4032515PMC
July 2014

Quality matters: how does mitochondrial network dynamics and quality control impact on mtDNA integrity?

Philos Trans R Soc Lond B Biol Sci 2014 Jul;369(1646):20130442

Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders, Radboud University Medical Centre, Geert Grooteplein 10, PO Box 9101, 6500 HB Nijmegen, The Netherlands FinMIT Centre of Excellence, Institute of Biomedical Technology and Tampere University Hospital, Pirkanmaa Hospital District, 33014 Tampere, Finland

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http://dx.doi.org/10.1098/rstb.2013.0442DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4032518PMC
July 2014

To be or not to be a nucleoid protein: a comparison of mass-spectrometry based approaches in the identification of potential mtDNA-nucleoid associated proteins.

Biochimie 2014 May 25;100:219-26. Epub 2013 Sep 25.

Department of Pediatrics, Nijmegen Centre for Mitochondrial Disorders, Radboud University Nijmegen Medical Centre, Geert Grooteplein 10, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands; Institute of Biomedical Technology & Tampere University Hospital, Pirkanmaa Hospital District, University of Tampere, FI-33014, Finland. Electronic address:

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http://dx.doi.org/10.1016/j.biochi.2013.09.017DOI Listing
May 2014

Analysis of 953 human proteins from a mitochondrial HEK293 fraction by complexome profiling.

PLoS One 2013 23;8(7):e68340. Epub 2013 Jul 23.

Department of Laboratory Medicine, Laboratory of Genetic Endocrine and Metabolic Disorders, Radboud University Medical Centre, Nijmegen, The Netherlands.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0068340PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3720734PMC
March 2014

Replication factors transiently associate with mtDNA at the mitochondrial inner membrane to facilitate replication.

Nucleic Acids Res 2014 Jan 25;42(2):952-67. Epub 2013 Oct 25.

FinMIT Centre of Excellence, Institute of Biomedical Technology & Tampere University Hospital, Pirkanmaa Hospital District, FI-33014 University of Tampere, Finland and Department of Pediatrics, Institute for Genetic and Metabolic Disease, Radboud University Medical Centre, Geert Grooteplein 10, P.O. Box 9101, 6500 HB, Nijmegen, The Netherlands.

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http://dx.doi.org/10.1093/nar/gkt988DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3902951PMC
January 2014

Mytoe: automatic analysis of mitochondrial dynamics.

Bioinformatics 2012 Apr 9;28(7):1050-1. Epub 2012 Feb 9.

Department of Signal Processing, Tampere University of Technology, FI-33520 Tampere, Finland.

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http://dx.doi.org/10.1093/bioinformatics/bts073DOI Listing
April 2012

Replication stalling by catalytically impaired Twinkle induces mitochondrial DNA rearrangements in cultured cells.

Mitochondrion 2011 Jul 20;11(4):630-4. Epub 2011 Apr 20.

Department of Cardiac Development and Remodelling, Max-Planck-Institute for Heart and Lung Research, Ludwigstrasse 43, 61231 Bad Nauheim, Germany.

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http://dx.doi.org/10.1016/j.mito.2011.04.002DOI Listing
July 2011

Functional organization of mammalian mitochondrial DNA in nucleoids: history, recent developments, and future challenges.

IUBMB Life 2010 Jan;62(1):19-32

FinMIT Centre of Excellence, Institute of Medical Technology and Tampere University Hospital, University of Tampere, Tampere 33014 TAY, Finland.

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http://dx.doi.org/10.1002/iub.282DOI Listing
January 2010

Human Dna2 is a nuclear and mitochondrial DNA maintenance protein.

Mol Cell Biol 2009 Aug 1;29(15):4274-82. Epub 2009 Jun 1.

Department of Cell Biology and Physiology, Washington University School of Medicine, 660 South Euclid Avenue, St. Louis, MO 63110, USA.

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http://mcb.asm.org/content/29/15/4274.full.pdf
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http://mcb.asm.org/cgi/doi/10.1128/MCB.01834-08
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http://dx.doi.org/10.1128/MCB.01834-08DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2715806PMC
August 2009

A new splice variant of the mouse SIRT3 gene encodes the mitochondrial precursor protein.

PLoS One 2009 31;4(3):e4986. Epub 2009 Mar 31.

University of Tampere, Institute of Medical Technology and Tampere University Hospital, Tampere, Finland.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0004986PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2659428PMC
May 2009

The human SIRT3 protein deacetylase is exclusively mitochondrial.

Biochem J 2008 Apr;411(2):279-85

Institute of Medical Technology and Tampere University Hospital, Tampere, Finland.

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http://dx.doi.org/10.1042/BJ20071624DOI Listing
April 2008

What causes mitochondrial DNA deletions in human cells?

Nat Genet 2008 Mar;40(3):275-9

Mitochondrial Research Group, The Medical School, Newcastle University, Newcastle upon Tyne, NE2 4HH, UK.

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http://dx.doi.org/10.1038/ng.f.94DOI Listing
March 2008

The mitochondria of cultured mammalian cells: I. Analysis by immunofluorescence microscopy, histochemistry, subcellular fractionation, and cell fusion.

Methods Mol Biol 2007 ;372:3-16

INSERM U582-Institut de Myologie, Université Pierre et Marie Curie, IFR14, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.

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http://dx.doi.org/10.1007/978-1-59745-365-3_1DOI Listing
March 2008

The mitochondria of cultured mammalian cells: II. Expression and visualization of exogenous proteins in fixed and live cells.

Methods Mol Biol 2007 ;372:17-32

FinMIT Centre of Excellence, Institute of Medical Technology and Tampere University Hospital, University of Tampere, Finland.

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http://dx.doi.org/10.1007/978-1-59745-365-3_2DOI Listing
March 2008

Twinkle helicase (PEO1) gene mutation causes mitochondrial DNA depletion.

Ann Neurol 2007 Dec;62(6):579-87

Institut National de la Sante et de la Recherche Médicale U781 and Service de Génétique, Hôpital Necker-Enfants Malades, Paris, France.

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http://dx.doi.org/10.1002/ana.21207DOI Listing
December 2007

Mammalian mitochondrial nucleoids: organizing an independently minded genome.

Mitochondrion 2007 Sep 4;7(5):311-21. Epub 2007 Jul 4.

MRC-Dunn Human Nutrition Unit, Wellcome Trust-MRC Building, Hills Road, Cambridge CB2 OXY, UK.

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http://dx.doi.org/10.1016/j.mito.2007.06.004DOI Listing
September 2007

Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes.

Nucleic Acids Res 2007 22;35(10):3238-51. Epub 2007 Apr 22.

Institute of Medical Technology and Tampere University Hospital, Tampere, Finland.

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http://dx.doi.org/10.1093/nar/gkm215DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1904276PMC
June 2007

Tid1 isoforms are mitochondrial DnaJ-like chaperones with unique carboxyl termini that determine cytosolic fate.

J Biol Chem 2006 May 10;281(19):13150-8. Epub 2006 Mar 10.

University of Medicine and Dentistry of New Jersey-New Jersey Medical School, Department of Biochemistry and Molecular Biology, Newark, New Jersey 07103, USA.

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http://dx.doi.org/10.1074/jbc.M509179200DOI Listing
May 2006

Mutant mitochondrial helicase Twinkle causes multiple mtDNA deletions and a late-onset mitochondrial disease in mice.

Proc Natl Acad Sci U S A 2005 Dec 21;102(49):17687-92. Epub 2005 Nov 21.

Department of Neurology and Programme of Neurosciences, University of Helsinki, 00290, Helsinki, Finland.

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http://dx.doi.org/10.1073/pnas.0505551102DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1308896PMC
December 2005

Infantile onset spinocerebellar ataxia is caused by recessive mutations in mitochondrial proteins Twinkle and Twinky.

Hum Mol Genet 2005 Oct 31;14(20):2981-90. Epub 2005 Aug 31.

Department of Molecular Medicine, National Public Health Institute, University of Helsinki, Finland.

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http://dx.doi.org/10.1093/hmg/ddi328DOI Listing
October 2005

Twinkle helicase is essential for mtDNA maintenance and regulates mtDNA copy number.

Hum Mol Genet 2004 Dec 27;13(24):3219-27. Epub 2004 Oct 27.

Department of Neurology and Programme of Neurosciences, University of Helsinki, 00290 Helsinki, Finland.

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http://dx.doi.org/10.1093/hmg/ddh342DOI Listing
December 2004

Twinkle and POLG defects enhance age-dependent accumulation of mutations in the control region of mtDNA.

Nucleic Acids Res 2004 4;32(10):3053-64. Epub 2004 Jun 4.

Institute of Medical Technology and Tampere University Hospital, Tampere, Finland.

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http://dx.doi.org/10.1093/nar/gkh634DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC434440PMC
June 2004

Ditercalinium chloride, a pro-anticancer drug, intimately associates with mammalian mitochondrial DNA and inhibits its replication.

Curr Genet 2003 Aug 5;43(5):364-70. Epub 2003 Apr 5.

Department of Clinical Chemistry and Laboratory Medicine, Kyushu University Graduate School of Medical Sciences, 3-1-1 Maidashi, Higashi-ku, 812-8582 Fukuoka, Japan.

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http://dx.doi.org/10.1007/s00294-003-0393-4DOI Listing
August 2003

Composition and dynamics of human mitochondrial nucleoids.

Mol Biol Cell 2003 Apr;14(4):1583-96

Institute of Medical Technology and Tampere University Hospital, Lenkkeilijänkatu 6, 33014 University of Tampere, Tampere, Finland.

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http://www.molbiolcell.org/doi/10.1091/mbc.e02-07-0399
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http://dx.doi.org/10.1091/mbc.e02-07-0399DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC153124PMC
April 2003

The 7472insC mitochondrial DNA mutation impairs the synthesis and extent of aminoacylation of tRNASer(UCN) but not its structure or rate of turnover.

J Biol Chem 2002 Jun 27;277(25):22240-50. Epub 2002 Mar 27.

Institute of Medical Technology and Tampere University Hospital, FIN-33014 University of Tampere, Finland.

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http://dx.doi.org/10.1074/jbc.M200338200DOI Listing
June 2002