Publications by authors named "Zofia M Chrzanowska-Lightowlers"

65Publications

Redecorating the Mitochondrial Inner Membrane: A Treatment for mtDNA Disorders.

Mol Ther 2020 08 16;28(8):1749-1751. Epub 2020 Jul 16.

Newcastle University Biosciences Institute, Wellcome Centre for Mitochondrial Research, Faculty of Medical Sciences, Newcastle upon Tyne, UK. Electronic address:

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http://dx.doi.org/10.1016/j.ymthe.2020.07.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7403454PMC
August 2020

Messenger RNA delivery to mitoribosomes - hints from a bacterial toxin.

FEBS J 2020 Apr 24. Epub 2020 Apr 24.

The Wellcome Centre for Mitochondrial Research, Institute of Neuroscience, Newcastle University, UK.

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http://dx.doi.org/10.1111/febs.15342DOI Listing
April 2020

Rescuing stalled mammalian mitoribosomes - what can we learn from bacteria?

J Cell Sci 2020 01 2;133(1). Epub 2020 Jan 2.

The Wellcome Centre for Mitochondrial Research, Newcastle University, The Medical School, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK

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http://dx.doi.org/10.1242/jcs.231811DOI Listing
January 2020

Biallelic Mutations in MTPAP Associated with a Lethal Encephalopathy.

Neuropediatrics 2020 06 28;51(3):178-184. Epub 2019 Nov 28.

Laboratory of Neurogenetics and Neuroinflammation, Sorbonne-Paris-Cité, Institut Imagine, Paris Descartes University, Paris, France.

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http://dx.doi.org/10.1055/s-0039-3400979DOI Listing
June 2020

Mammalian mitochondrial translation - revealing consequences of divergent evolution.

Biochem Soc Trans 2019 10;47(5):1429-1436

The Wellcome Centre for Mitochondrial Research, Institute of Neuroscience, Newcastle University, Newcastle upon Tyne NE2 4HH, U.K.

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http://dx.doi.org/10.1042/BST20190265DOI Listing
October 2019

Defective mitochondrial protease LonP1 can cause classical mitochondrial disease.

Hum Mol Genet 2018 05;27(10):1743-1753

Wellcome Centre for Mitochondrial Research, Institute for Cell and Molecular Biosciences, The Medical School, Newcastle University, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1093/hmg/ddy080DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5932559PMC
May 2018

Using mitoribosomal profiling to investigate human mitochondrial translation.

Wellcome Open Res 2017 11;2:116. Epub 2017 Dec 11.

The Wellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, Newcastle University, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.12688/wellcomeopenres.13119.2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5771143PMC
December 2017

The human RNA-binding protein RBFA promotes the maturation of the mitochondrial ribosome.

Biochem J 2017 06 13;474(13):2145-2158. Epub 2017 Jun 13.

Institute for Cell and Molecular Bioscience, Newcastle University, Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, U.K.

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http://dx.doi.org/10.1042/BCJ20170256DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5468982PMC
June 2017

Human mitochondrial nucleases.

FEBS J 2017 06 1;284(12):1767-1777. Epub 2017 Feb 1.

The Wellcome Trust Centre for Mitochondrial Research, The Medical School, Newcastle University, UK.

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http://dx.doi.org/10.1111/febs.13981DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5484287PMC
June 2017

Autophagy impairment with lysosomal and mitochondrial dysfunction is an important characteristic of oxidative stress-induced senescence.

Autophagy 2017 Jan 28;13(1):99-113. Epub 2016 Oct 28.

a Lab for Aging Research, Center of Gerontology and Geriatrics, State Key Laboratory of Biotherapy & Collaborative Innovation Center of Biotherapy, West China Hospital, Sichuan University , Chengdu , China.

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https://www.tandfonline.com/doi/full/10.1080/15548627.2016.1
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http://dx.doi.org/10.1080/15548627.2016.1247143DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5240829PMC
January 2017

Human mitochondrial ribosomes can switch their structural RNA composition.

Proc Natl Acad Sci U S A 2016 10 11;113(43):12198-12201. Epub 2016 Oct 11.

The Wellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, Newcastle University, Newcastle upon Tyne NE2 4HH, United Kingdom; The Wellcome Trust Centre for Mitochondrial Research, Institute for Cell and Molecular Biosciences, Newcastle University, Newcastle upon Tyne NE2 4HH, United Kingdom

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http://dx.doi.org/10.1073/pnas.1609338113DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5087001PMC
October 2016

The process of mammalian mitochondrial protein synthesis.

Cell Tissue Res 2017 Jan 14;367(1):5-20. Epub 2016 Jul 14.

Wellcome Trust Centre for Mitochondrial Research, Institute for Cell and Molecular Biosciences, Medical School, Newcastle University, Newcastle upon Tyne, NE2 4HH, UK.

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http://dx.doi.org/10.1007/s00441-016-2456-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5203842PMC
January 2017

SLIRP stabilizes LRPPRC via an RRM-PPR protein interface.

Nucleic Acids Res 2016 08 28;44(14):6868-82. Epub 2016 Jun 28.

Department of Mitochondrial Biology, Max Planck Institute for Biology of Ageing, D-50931 Cologne, Germany Department of Medical Biochemistry and Biophysics, Karolinska Institutet, 171 77 Stockholm, Sweden

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http://dx.doi.org/10.1093/nar/gkw575DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5001613PMC
August 2016

The presence of highly disruptive 16S rRNA mutations in clinical samples indicates a wider role for mutations of the mitochondrial ribosome in human disease.

Mitochondrion 2015 Nov 5;25:17-27. Epub 2015 Sep 5.

Grupo GIBE, Bioloxía Celular e Molecular, Facultade de Ciencias, Universidade da Coruña (UDC), Campus Zapateira s/n, 15071 A Coruña, Spain. Electronic address:

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http://dx.doi.org/10.1016/j.mito.2015.08.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4665369PMC
November 2015

Overcoming stalled translation in human mitochondria.

Front Microbiol 2014 18;5:374. Epub 2014 Jul 18.

Wellcome Trust Centre for Mitochondrial Research, Institute for Cell and Molecular Biosciences, Newcastle University, Medical School Newcastle upon Tyne, UK.

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http://dx.doi.org/10.3389/fmicb.2014.00374DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4103422PMC
August 2014

Mitochondrial protein synthesis: figuring the fundamentals, complexities and complications, of mammalian mitochondrial translation.

FEBS Lett 2014 Aug 6;588(15):2496-503. Epub 2014 Jun 6.

The Wellcome Trust Centre for Mitochondrial Research, Institute of Neuroscience, Newcastle University, The Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, UK. Electronic address:

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http://dx.doi.org/10.1016/j.febslet.2014.05.054DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4099522PMC
August 2014

Salvaging hope: Is increasing NAD(+) a key to treating mitochondrial myopathy?

EMBO Mol Med 2014 Jun 6;6(6):705-7. Epub 2014 Apr 6.

Wellcome Trust Centre for Mitochondrial Research, Institute for Cell and Molecular Biosciences Medical School Newcastle University, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.15252/emmm.201404179DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4203349PMC
June 2014

Human mitochondrial leucyl tRNA synthetase can suppress non cognate pathogenic mt-tRNA mutations.

EMBO Mol Med 2014 02 10;6(2):183-93. Epub 2014 Jan 10.

The Wellcome Trust Centre for Mitochondrial Research Institute for Ageing and Health The Medical School, Newcastle University, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1002/emmm.201303202DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3927954PMC
February 2014

Mutation of the human mitochondrial phenylalanine-tRNA synthetase causes infantile-onset epilepsy and cytochrome c oxidase deficiency.

Biochim Biophys Acta 2014 Jan 24;1842(1):56-64. Epub 2013 Oct 24.

Wellcome Trust Centre for Mitochondrial Research, Institute for Ageing and Health, Newcastle University, Newcastle upon Tyne NE2 4HH, UK. Electronic address:

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http://dx.doi.org/10.1016/j.bbadis.2013.10.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3898479PMC
January 2014

Interactions between peptidyl tRNA hydrolase homologs and the ribosomal release factor Mrf1 in S. pombe mitochondria.

Mitochondrion 2013 Nov 24;13(6):871-80. Epub 2013 Jul 24.

Centre de Génétique Moléculaire, UPR3404, FRC3115, Avenue de la Terrasse, 91198 Gif-sur-Yvette Cedex, France.

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http://dx.doi.org/10.1016/j.mito.2013.07.115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3919214PMC
November 2013

REXO2 is an oligoribonuclease active in human mitochondria.

PLoS One 2013 31;8(5):e64670. Epub 2013 May 31.

The Wellcome Trust Centre for Mitochondrial Research, Institute for Ageing and Health, Newcastle University, The Medical School, Newcastle upon Tyne, United Kingdom.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0064670PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3669425PMC
February 2014

Human pentatricopeptide proteins: only a few and what do they do?

RNA Biol 2013 23;10(9):1433-8. Epub 2013 Apr 23.

The Wellcome Trust Centre for Mitochondrial Research; Institute for Ageing and Health; Newcastle University; The Medical School; Framlington Place; Newcastle upon Tyne, UK.

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http://dx.doi.org/10.4161/rna.24770DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3858426PMC
May 2015

Mutations in mitochondrial ribosomal protein MRPL12 leads to growth retardation, neurological deterioration and mitochondrial translation deficiency.

Biochim Biophys Acta 2013 Aug 18;1832(8):1304-12. Epub 2013 Apr 18.

Université Paris Descartes-Sorbonne Paris Cité, Institut Imagine and INSERM U781, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75015 Paris, France.

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http://dx.doi.org/10.1016/j.bbadis.2013.04.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3787750PMC
August 2013

GRSF1 regulates RNA processing in mitochondrial RNA granules.

Cell Metab 2013 Mar;17(3):399-410

Department of Cell Biology, University of Geneva, 30 Quai Ernest-Ansermet, 1211 Genève 4, Switzerland.

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http://dx.doi.org/10.1016/j.cmet.2013.02.005DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3593211PMC
March 2013

The mystery of mitochondrial RNases.

Biochem Soc Trans 2012 Aug;40(4):865-9

Wellcome Trust Centre for Mitochondrial Research, Institute for Ageing and Health, Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1042/BST20120022DOI Listing
August 2012

Structure based hypothesis of a mitochondrial ribosome rescue mechanism.

Biol Direct 2012 May 8;7:14. Epub 2012 May 8.

Centre for Molecular and Biomolecular Informatics, Nijmegen Centre for Molecular Life Sciences, Radboud University Nijmegen Medical Centre, P,O, Box 9101, 6400, HB Nijmegen, The Netherlands.

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http://dx.doi.org/10.1186/1745-6150-7-14DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3418547PMC
May 2012

Recombinant human MDM2 oncoprotein shows sequence composition selectivity for binding to both RNA and DNA.

Int J Oncol 2012 Mar 21;40(3):851-9. Epub 2011 Nov 21.

Northern Institute for Cancer Research, Newcastle University Medical School, Newcastle-upon-Tyne, UK.

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http://dx.doi.org/10.3892/ijo.2011.1267DOI Listing
March 2012

Termination of protein synthesis in mammalian mitochondria.

J Biol Chem 2011 Oct 26;286(40):34479-85. Epub 2011 Aug 26.

Mitochondrial Research Group, Institute for Ageing and Health, Newcastle University, Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, United Kingdom.

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http://dx.doi.org/10.1074/jbc.R111.290585DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3186377PMC
October 2011

Translation termination in human mitochondrial ribosomes.

Biochem Soc Trans 2010 Dec;38(6):1523-6

Mitochondrial Research Group, Institute for Ageing and Health, Newcastle University Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, UK.

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http://dx.doi.org/10.1042/BST0381523DOI Listing
December 2010

Terminating human mitochondrial protein synthesis: a shift in our thinking.

RNA Biol 2010 May-Jun;7(3):282-6. Epub 2010 May 8.

Institute for Ageing and Health, Newcastle University, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.4161/rna.7.3.12023DOI Listing
February 2011

Human mitochondrial mRNAs--like members of all families, similar but different.

Biochim Biophys Acta 2010 Jun-Jul;1797(6-7):1081-5. Epub 2010 Mar 6.

Mitochondrial Research Group, Medical School, Institute for Ageing and Health, Newcastle University, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK.

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https://linkinghub.elsevier.com/retrieve/pii/S00052728100009
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http://dx.doi.org/10.1016/j.bbabio.2010.02.036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3003153PMC
January 2011

Differences in RNA processing underlie the tissue specific phenotype of ISCU myopathy.

Biochim Biophys Acta 2010 Jun 4;1802(6):539-44. Epub 2010 Mar 4.

Department of Clinical Medicine, University of Bergen, Norway.

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http://dx.doi.org/10.1016/j.bbadis.2010.02.010DOI Listing
June 2010

A functional peptidyl-tRNA hydrolase, ICT1, has been recruited into the human mitochondrial ribosome.

EMBO J 2010 Mar 25;29(6):1116-25. Epub 2010 Feb 25.

Mitochondrial Research Group, Institute for Ageing and Health, Medical School, Newcastle University, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1038/emboj.2010.14DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2845271PMC
March 2010

Targeting of the cytosolic poly(A) binding protein PABPC1 to mitochondria causes mitochondrial translation inhibition.

Nucleic Acids Res 2010 Jun 9;38(11):3732-42. Epub 2010 Feb 9.

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

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http://dx.doi.org/10.1093/nar/gkq068DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2887948PMC
June 2010

Hungry codons promote frameshifting in human mitochondrial ribosomes.

Science 2010 Jan;327(5963):301

The Mitochondrial Research Group, Institute for Ageing and Health, Newcastle University, Framlington Place, Newcastle upon Tyne NE2 4HH, UK.

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http://dx.doi.org/10.1126/science.1180674DOI Listing
January 2010

Fine mapping of interactions between eEF1alpha protein and 3'UTR of metallothionein-1 mRNA.

Biochem Biophys Res Commun 2009 Aug 6;386(1):82-8. Epub 2009 Jun 6.

Institute for Cell and Molecular Biosciences, Newcastle University, The Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, UK.

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http://dx.doi.org/10.1016/j.bbrc.2009.05.146DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2719692PMC
August 2009

PPR (pentatricopeptide repeat) proteins in mammals: important aids to mitochondrial gene expression.

Biochem J 2008 Nov;416(1):e5-6

Mitochondrial Research Group, Newcastle University, The Medical School, Framlington Place, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1042/BJ20081942DOI Listing
November 2008

The human mitochondrial ribosome recycling factor is essential for cell viability.

Nucleic Acids Res 2008 Oct 9;36(18):5787-99. Epub 2008 Sep 9.

Mitochondrial Research Group, Institute of Cellular Medicine, Medical School, Newcastle University, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK.

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http://dx.doi.org/10.1093/nar/gkn576DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2566884PMC
October 2008

Production of transmitochondrial cybrids containing naturally occurring pathogenic mtDNA variants.

Nucleic Acids Res 2006 Aug 2;34(13):e95. Epub 2006 Aug 2.

Mitochondrial Research Group, University of Newcastle upon Tyne, School of Neurology, Neurobiology and Psychiatry, Framlington Place, Newcastle upon Tyne NE2 4HH, UK.

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http://dx.doi.org/10.1093/nar/gkl516DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1540737PMC
August 2006

Serum-deprivation stimulates cap-binding by PARN at the expense of eIF4E, consistent with the observed decrease in mRNA stability.

Nucleic Acids Res 2005 14;33(1):376-87. Epub 2005 Jan 14.

Department of Neurology, The Medical School, University of Newcastle upon Tyne, Framlington Place, Newcastle upon Tyne, NE2 4HH, UK.

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https://academic.oup.com/nar/article-lookup/doi/10.1093/nar/
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http://dx.doi.org/10.1093/nar/gki169DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC546156PMC
February 2005

Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells.

Hum Mol Genet 2004 Oct 18;13(20):2519-34. Epub 2004 Aug 18.

CNRS-FRE 2375, Institute of Physiology and Biological Chemistry, Université Louis Pasteur, 21 René Descartes, 67084 Strasbourg, France.

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http://dx.doi.org/10.1093/hmg/ddh267DOI Listing
October 2004

Sporadic mitochondrial myopathy due to a new mutation in the mitochondrial tRNASer(UCN) gene.

Neuromuscul Disord 2004 Jul;14(7):417-20

Mitochondrial Research Group, School of Neurology, Neurobiology and Psychiatry, The Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, UK.

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http://dx.doi.org/10.1016/j.nmd.2004.03.004DOI Listing
July 2004

Messenger RNA stability in mitochondria: different means to an end.

Trends Genet 2004 Jun;20(6):260-7

IBMP CNRS, 12 rue du Général Zimmer, 67084 Strasbourg Cedex, France.

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http://dx.doi.org/10.1016/j.tig.2004.04.006DOI Listing
June 2004

Functional polypeptides can be synthesized from human mitochondrial transcripts lacking termination codons.

Biochem J 2004 Feb;377(Pt 3):725-31

School of Neurology, The Medical School, University of Newcastle upon Tyne, Framlington Place, Newcastle upon Tyne, NE2 4HH, U.K.

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http://biochemj.org/lookup/doi/10.1042/bj20031556
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http://dx.doi.org/10.1042/BJ20031556DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1223913PMC
February 2004

Investigation of a pathogenic mtDNA microdeletion reveals a translation-dependent deadenylation decay pathway in human mitochondria.

Hum Mol Genet 2003 Sep 22;12(18):2341-8. Epub 2003 Jul 22.

Medical School, University of Newcastle upon Tyne, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1093/hmg/ddg238DOI Listing
September 2003

A splice junction mutation in muscle carnitine palmitoyltransferase II deficiency.

Mol Genet Metab 2003 Jun;79(2):124-8

Department of Neurology, Martin-Luther-University of Halle-Wittenberg, Ernst-Grube-Str. 40, 06097, Halle/Saale, Germany.

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http://dx.doi.org/10.1016/s1096-7192(03)00067-2DOI Listing
June 2003

Mu class glutathione S-transferase mRNA isoform expression in acute lymphoblastic leukaemia.

Br J Haematol 2003 Jan;120(1):80-8

Department of Paediatric Oncology, University of Bristol, UK.

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http://dx.doi.org/10.1046/j.1365-2141.2003.04039.xDOI Listing
January 2003