Ian J Holt

Ian J Holt

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Ian J Holt

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The mitochondrial R-loop.

Authors:
Ian J Holt

Nucleic Acids Res 2019 Jun;47(11):5480-5489

Biodonostia Health Research Institute, 20014 San Sebastián, Spain & IKERBASQUE, Basque Foundation for Science, 48013 Bilbao, Spain.

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http://dx.doi.org/10.1093/nar/gkz277DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6582354PMC
June 2019

The Jekyll and Hyde character of RNase H1 and its multiple roles in mitochondrial DNA metabolism.

Authors:
Ian J Holt

DNA Repair (Amst) 2019 Jun 4:102630. Epub 2019 Jun 4.

Biodonostia Health Research Institute, 20014 San Sebastián, Spain; IKERBASQUE, Basque Foundation for Science, 48013 Bilbao, Spain; Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, Royal Free Campus, London, NW3 2PF, UK; CIBERNED (Center for Networked Biomedical Research on Neurodegenerative Diseases, Ministry of Economy and Competitiveness, Institute Carlos III), Madrid, Spain. Electronic address:

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http://dx.doi.org/10.1016/j.dnarep.2019.06.001DOI Listing
June 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

Reply: Genotype-phenotype correlation in ATAD3A deletions: not just of scientific relevance.

Brain 2017 11;140(11):e67

Murdoch Children's Research Institute, Royal Children's Hospital and Department of Paediatrics, University of Melbourne, Melbourne VIC 3052, Australia.

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http://dx.doi.org/10.1093/brain/awx240DOI Listing
November 2017

Clinicopathologic and molecular spectrum of -related mitochondrial disease.

Neurol Genet 2017 Jun 2;3(3):e149. Epub 2017 May 2.

MRC Centre for Neuromuscular Diseases (E.B., O.V.P., A.M., A.H., J.L.H., H.H., M.G.H., R.D.S.P.), UCL Institute of Neurology and National Hospital for Neurology and Neurosurgery; Department of Molecular Neuroscience (A.M., A.M.P., J.L.H., H.H., M.G.H.), Division of Neuropathology (J.L.H.), Department of Clinical Neuroscience (J.-W.T., A.S., I.J.H.), UCL Institute of Neurology; Neurometabolic Unit (I.H.), Neurogenetics Unit (C.E.W., M.G.S.), Department of Neuro-ophthalmology (G.T.P.), National Hospital for Neurology and Neurosurgery, London; Nuffield Department of Obstetrics and Gynaecology (J.P.), University of Oxford; MRC-Mitochondrial Biology Unit (M.Z.), Cambridge, UK; Unit of Molecular Neurogenetics (D.G.), Fondazione IRCCS Istituto Neurologico "Carlo Besta," Milan, Italy; Oxford Medical Genetics Laboratories (J.T., C.S., C.F.), Oxford University Hospitals NHS Foundation Trust, Churchill Hospital, UK; Department of Neurology (M.A.K.), Nizam's Institute of Medical Sciences; CSIR-Centre for Cellular and Molecular Biology (A.P., K.T.), Hyderabad, Telangana, India; MRC Mill Hill Laboratory (I.J.H.), London, UK; Biodonostia Research Institute (I.J.H.), San Sebastián, Spain; and Department of Basic and Clinical Neuroscience (R.D.S.P.), Institute of Psychiatry, Psychology and Neuroscience, King's College London, UK.

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http://dx.doi.org/10.1212/NXG.0000000000000149DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5413961PMC
June 2017

Analysis of Replicating Mitochondrial DNA by In Organello Labeling and Two-Dimensional Agarose Gel Electrophoresis.

Methods Mol Biol 2016 ;1351:95-113

MRC-Mitochondrial Biology Unit, Hills Road, Cambridge, CB2 0XY, UK.

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http://dx.doi.org/10.1007/978-1-4939-3040-1_8DOI Listing
September 2016

A robust assay to measure DNA topology-dependent protein binding affinity.

Nucleic Acids Res 2015 Apr 30;43(7):e43. Epub 2014 Dec 30.

National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20814, USA

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http://dx.doi.org/10.1093/nar/gku1381DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4402506PMC
April 2015

Unique features of DNA replication in mitochondria: a functional and evolutionary perspective.

Bioessays 2014 Nov 12;36(11):1024-31. Epub 2014 Sep 12.

MRC National Institute for Medical Research, London, UK.

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http://dx.doi.org/10.1002/bies.201400052DOI Listing
November 2014

The road to rack and ruin: selecting deleterious mitochondrial DNA variants.

Philos Trans R Soc Lond B Biol Sci 2014 Jul;369(1646):20130451

Institute for Ageing and Health, Newcastle University, Campus for Ageing and Vitality, Newcastle upon Tyne NE4 5PL, UK.

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http://dx.doi.org/10.1098/rstb.2013.0451DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4032523PMC
July 2014

Mitochondrial transcription terminator family members mTTF and mTerf5 have opposing roles in coordination of mtDNA synthesis.

PLoS Genet 2013 19;9(9):e1003800. Epub 2013 Sep 19.

Institute of Biomedical Technology and Tampere University Hospital, Tampere, Finland ; Estonian Biocentre, Tartu, Estonia.

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http://dx.doi.org/10.1371/journal.pgen.1003800DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3778013PMC
March 2014

Mitochondrial DNA replication proceeds via a 'bootlace' mechanism involving the incorporation of processed transcripts.

Nucleic Acids Res 2013 Jun 16;41(11):5837-50. Epub 2013 Apr 16.

MRC-Mitochondrial Biology Unit, Wellcome Trust/MRC Building, Hills Road, Cambridge CB2 0XY, UK.

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http://dx.doi.org/10.1093/nar/gkt196DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3675460PMC
June 2013

Alternative translation initiation augments the human mitochondrial proteome.

Nucleic Acids Res 2013 Feb 28;41(4):2354-69. Epub 2012 Dec 28.

MRC-Mitochondrial Biology Unit, Wellcome Trust-MRC Building, Cambridge CB2 0XY, UK.

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http://dx.doi.org/10.1093/nar/gks1347DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3575844PMC
February 2013

Human mitochondrial DNA replication.

Cold Spring Harb Perspect Biol 2012 Dec 1;4(12). Epub 2012 Dec 1.

MRC Mitochondrial Biology Unit, Cambridge, United Kingdom.

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http://dx.doi.org/10.1101/cshperspect.a012971DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3504440PMC
December 2012

Minimizing the damage: repair pathways keep mitochondrial DNA intact.

Nat Rev Mol Cell Biol 2012 Oct 20;13(10):659-71. Epub 2012 Sep 20.

MRC-Mitochondrial Biology Unit, Wellcome Trust/MRC Building, Cambridge, UK.

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http://dx.doi.org/10.1038/nrm3439DOI Listing
October 2012

Human telomerase acts as a hTR-independent reverse transcriptase in mitochondria.

Nucleic Acids Res 2012 Jan 21;40(2):712-25. Epub 2011 Sep 21.

Department of Pharmacology and Physiology, New Jersey Medical School of UMDNJ, 185 South Orange Avenue, Medical Sciences Building, Newark, NJ 07103, USA.

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http://dx.doi.org/10.1093/nar/gkr758DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3258147PMC
January 2012

TEFM (c17orf42) is necessary for transcription of human mtDNA.

Nucleic Acids Res 2011 May 28;39(10):4284-99. Epub 2011 Jan 28.

MRC Mitochondrial Biology Unit, Wellcome Trust/MRC Building, Hills Road, Cambridge CB2 0XY, UK.

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http://dx.doi.org/10.1093/nar/gkq1224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3105396PMC
May 2011

Overexpression of MTERFD1 or MTERFD3 impairs the completion of mitochondrial DNA replication.

Mol Biol Rep 2011 Feb 25;38(2):1321-8. Epub 2010 Jun 25.

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

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http://dx.doi.org/10.1007/s11033-010-0233-9DOI Listing
February 2011

An upstream open reading frame and the context of the two AUG codons affect the abundance of mitochondrial and nuclear RNase H1.

Mol Cell Biol 2010 Nov 7;30(21):5123-34. Epub 2010 Sep 7.

Program in Genomics of Differentiation, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, MD 20892, USA.

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http://dx.doi.org/10.1128/MCB.00619-10DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2953059PMC
November 2010

Transmission of mitochondrial DNA diseases and ways to prevent them.

PLoS Genet 2010 Aug 12;6(8). Epub 2010 Aug 12.

Nuffield Department of Obstetrics and Gynaecology, University of Oxford, Oxford, UK.

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http://dx.doi.org/10.1371/journal.pgen.1001066DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2920841PMC
August 2010

Zen and the art of mitochondrial DNA maintenance.

Authors:
Ian J Holt

Trends Genet 2010 Mar 1;26(3):103-9. Epub 2010 Feb 1.

MRC Mitochondrial Biology Unit, Wellcome Trust MRC Building Hills Rd, Cambridge, CB2 0XY, UK.

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http://dx.doi.org/10.1016/j.tig.2009.12.011DOI Listing
March 2010

Clinical and molecular aspects of diseases of mitochondrial DNA instability.

Chang Gung Med J 2009 Jul-Aug;32(4):354-69

Department of Anesthesiology, Chang Gung Memorial Hospital, Taipei, Chang Gung University College of Medicine, Taoyuan, Taiwan.

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November 2009

Analysis of mitochondrial DNA by two-dimensional agarose gel electrophoresis.

Methods Mol Biol 2009 ;554:15-35

MRC Mitochondrial Biology Unit, Wellcome Trust/MRC Building, Cambridge, UK.

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http://dx.doi.org/10.1007/978-1-59745-521-3_2DOI Listing
October 2009

Inhibition of mitochondrial fission favours mutant over wild-type mitochondrial DNA.

Hum Mol Genet 2009 Sep 27;18(18):3407-16. Epub 2009 Jun 27.

Neuroscience Department, University of Padova, Padova, Italy.

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http://dx.doi.org/10.1093/hmg/ddp281DOI Listing
September 2009

Mitochondrial DNA replication and repair: all a flap.

Authors:
Ian J Holt

Trends Biochem Sci 2009 Jul 24;34(7):358-65. Epub 2009 Jun 24.

MRC Mitochondrial Biology Unit, Cambridge, UK.

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http://dx.doi.org/10.1016/j.tibs.2009.03.007DOI Listing
July 2009

163rd ENMC International Workshop: nucleoid and nucleotide biology in syndromes of mitochondrial DNA depletion myopathy 12-14 December 2008, Naarden, The Netherlands.

Neuromuscul Disord 2009 Jun 21;19(6):439-43. Epub 2009 May 21.

The Women's Centre, John Radcliffe Hospital, Oxford, OX3 9DU, UK.

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http://dx.doi.org/10.1016/j.nmd.2009.04.009DOI Listing
June 2009

The conserved translocase Tim17 prevents mitochondrial DNA loss.

Hum Mol Genet 2009 Jan 30;18(1):65-74. Epub 2008 Sep 30.

Department of Molecular Biology, University of Texas Southwestern Medical Center, 6000 Harry Hines Boulevard, Dallas, TX 75390, USA.

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http://dx.doi.org/10.1093/hmg/ddn313DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3298863PMC
January 2009

The yeast Holliday junction resolvase, CCE1, can restore wild-type mitochondrial DNA to human cells carrying rearranged mitochondrial DNA.

Hum Mol Genet 2007 Oct 31;16(19):2306-14. Epub 2007 Jul 31.

MRC-Dunn Human Nutrition Unit, Cambridge, UK.

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http://dx.doi.org/10.1093/hmg/ddm182DOI Listing
October 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 algal nuclear ATP synthase subunit 6 in human cells results in protein targeting to mitochondria but no assembly into ATP synthase.

Rejuvenation Res 2006 ;9(4):455-69

MRC-Dunn Human Nutrition Unit, Cambridge, United Kingdom.

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http://dx.doi.org/10.1089/rej.2006.9.455DOI Listing
January 2007

Acquisition of the wobble modification in mitochondrial tRNALeu(CUN) bearing the G12300A mutation suppresses the MELAS molecular defect.

Hum Mol Genet 2006 Mar 30;15(6):897-904. Epub 2006 Jan 30.

Department of Chemistry and Biotechnology, Graduate School of Engineering, Graduate School of Frontier Sciences, University of Tokyo, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8656, Japan.

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http://dx.doi.org/10.1093/hmg/ddl007DOI Listing
March 2006

A bidirectional origin of replication maps to the major noncoding region of human mitochondrial DNA.

Mol Cell 2005 Jun;18(6):651-62

MRC-Dunn Human Nutrition Unit, Wellcome Trust-MRC Building, Hills Road, Cambridge CB2 2XY, United Kingdom.

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http://dx.doi.org/10.1016/j.molcel.2005.05.002DOI Listing
June 2005

Wobble modification deficiency in mutant tRNAs in patients with mitochondrial diseases.

FEBS Lett 2005 May;579(13):2948-52

Department of Chemistry and Biotechnology, Graduate School of Engineering, University of Tokyo, Japan.

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http://dx.doi.org/10.1016/j.febslet.2005.04.038DOI Listing
May 2005

Bidirectional replication initiates at sites throughout the mitochondrial genome of birds.

J Biol Chem 2005 Feb 20;280(5):3242-50. Epub 2004 Nov 20.

Medical Research Council Dunn Human Nutrition Unit, Wellcome Trust/MRC Building, Hills Road Cambridge, CB2 2XY, United Kingdom.

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http://dx.doi.org/10.1074/jbc.M411916200DOI Listing
February 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

Mammalian mitochondrial DNA replicates bidirectionally from an initiation zone.

J Biol Chem 2003 Dec 23;278(51):50961-9. Epub 2003 Sep 23.

Dunn Human Nutrition Unit, Wellcome Trust-Medical Research Council Building, Hills Road, Cambridge CB2 2XY, United Kingdom.

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http://dx.doi.org/10.1074/jbc.M308028200DOI Listing
December 2003

Response: The mitochondrial DNA replication bubble has not burst.

Trends Biochem Sci 2003 Jul;28(7):355-6

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http://dx.doi.org/10.1016/S0968-0004(03)00133-6DOI Listing
July 2003

Blue Native electrophoresis to study mitochondrial and other protein complexes.

Methods 2002 Apr;26(4):327-34

Nijmegen Centre for Mitochondrial Disorders, University Medical Centre Nijmegen, Geert Grooteplein 10, PO BOX 9101, 6500 HB Nijmegen, The Netherlands.

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http://linkinghub.elsevier.com/retrieve/pii/S104620230200038
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http://dx.doi.org/10.1016/S1046-2023(02)00038-5DOI Listing
April 2002