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Zofia Chrzanowska-Lightowlers

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Chemicals & Drugs

Anatomy

Physiology

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Mitochondrial Encephalomyopathies

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Francesco Bruni; Pasqua Gramegna; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
The mystery of mitochondrial RNases.
Martijn A Huynen; Zofia M A Chrzanowska-Lightowlers; Isabel Duarte; Sander B Nabuurs
Structure based hypothesis of a mitochondrial ribosome rescue mechanism.
Zofia M A Chrzanowska-Lightowlers; Aleksandra Pajak; Robert N Lightowlers
Termination of protein synthesis in mammalian mitochondria.

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All Publications

2012: Francesco Bruni; Pasqua Gramegna; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
The mystery of mitochondrial RNases.
Biochemical Society transactions 2012;40(4):865-9.
2012: Martijn A Huynen; Zofia M A Chrzanowska-Lightowlers; Isabel Duarte; Sander B Nabuurs
Structure based hypothesis of a mitochondrial ribosome rescue mechanism.
Biology direct 2012;7():14.
2011: Zofia M A Chrzanowska-Lightowlers; Aleksandra Pajak; Robert N Lightowlers
Termination of protein synthesis in mammalian mitochondria.
The Journal of biological chemistry 2011;286(40):34479-85.
2011: Zofia M A Chrzanowska-Lightowlers; R Horvath; Robert N Lightowlers
175th ENMC International Workshop: Mitochondrial protein synthesis in health and disease, 25-27th June 2010, Naarden, The Netherlands.
Neuromuscular disorders : NMD 2011;21(2):142-7.
2011: John P Kemp; Paul M Smith; Angela Pyle; Vivienne C M Neeve; Helen A L Tuppen; Ulrike Schara; Beril Talim; Haluk Topaloglu; Elke Holinski-Feder; Angela Abicht; Birgit Czermin; Hanns Lochmüller; Robert McFarland; Patrick F Chinnery; Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers; Robert W Taylor; Rita Horvath
Nuclear factors involved in mitochondrial translation cause a subgroup of combined respiratory chain deficiency.
Brain : a journal of neurology 2011;134(Pt 1):183-95.
2010: Ricarda Richter; Aleksandra Pajak; Sven Dennerlein; Agata Rozanska; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
Translation termination in human mitochondrial ribosomes.
Biochemical Society transactions 2010;38(6):1523-6.
2010: Andrew H Crosby; Heema Patel; Barry A Chioza; christos proukakis; Kay Gurtz; Michael A Patton; Reza Sharifi; Gaurav Harlalka; Michael A Simpson; Katherine Dick; Johanna A Reed; Ali Al-Memar; Zofia M A Chrzanowska-Lightowlers; Harold E Cross; Robert N Lightowlers
Defective mitochondrial mRNA maturation is associated with spastic ataxia.
American journal of human genetics 2010;87(5):655-60.
2010: Helen A L Tuppen; Vanessa E Hogan; Langping He; Emma L Blakely; Lisa Worgan; Mazhor Al-Dosary; Gabriele Saretzki; Charlotte L Alston; Andrew A Morris; Michael Clarke; Simon Jones; Anita M Devlin; Sahar Mansour; Zofia M A Chrzanowska-Lightowlers; David R Thorburn; Robert McFarland; Robert W Taylor
The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families.
Brain : a journal of neurology 2010;133(10):2952-63.
2010: Sven Dennerlein; Agata Rozanska; Mateusz Wydro; Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers
Human ERAL1 is a mitochondrial RNA chaperone involved in the assembly of the 28S small mitochondrial ribosomal subunit.
The Biochemical journal 2010;430(3):551-8.
2010: Petter S Sanaker; Marina Toompuu; Vanessa E Hogan; Langping He; Charalampos Tzoulis; Zofia M A Chrzanowska-Lightowlers; Robert W Taylor; Laurence A Bindoff
Differences in RNA processing underlie the tissue specific phenotype of ISCU myopathy.
Biochimica et biophysica acta 2010;1802(6):539-44.
2010: Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
Terminating human mitochondrial protein synthesis: a shift in our thinking.
RNA biology 2010;7(3):282-6.
2009: Rita Horvath; John P Kemp; Helen A L Tuppen; Gavin Hudson; Anders Oldfors; Suely K N Marie; Ali-Reza Moslemi; Serenella Servidei; Elisabeth Holme; Sara Shanske; Gittan Kollberg; Parul Jayakar; Angela Pyle; Harold M Marks; Elke Holinski-Feder; Mena Scavina; Maggie C Walter; Jorida Coku; Andrea Günther-Scholz; Paul M Smith; Robert McFarland; Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers; Michio Hirano; Hanns Lochmüller; Robert W Taylor; Patrick F Chinnery; Mar Tulinius; Salvatore DiMauro
Molecular basis of infantile reversible cytochrome c oxidase deficiency myopathy.
Brain : a journal of neurology 2009;132(Pt 11):3165-74.
2009: Kunbo Fan; Zofia M A Chrzanowska-Lightowlers; John Hesketh
Fine mapping of interactions between eEF1alpha protein and 3'UTR of metallothionein-1 mRNA.
Biochemical and biophysical research communications 2009;386(1):82-8.
2008: Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
PPR (pentatricopeptide repeat) proteins in mammals: important aids to mitochondrial gene expression.
The Biochemical journal 2008;416(1):e5-6.
2008: Joanna Rorbach; Ricarda Richter; Hans J Wessels; Mateusz Wydro; Marcin Lukasz Pekalski; Murtada Farhoud; Inge Kühl; Mauricette Gaisne; Nathalie Bonnefoy; Jan A M Smeitink; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
The human mitochondrial ribosome recycling factor is essential for cell viability.
Nucleic acids research 2008;36(18):5787-99.
2008: Agnieszka J Bobrowicz; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
Polyadenylation and degradation of mRNA in mammalian mitochondria: a missing link?
Biochemical Society transactions 2008;36(Pt 3):517-9.
2008: Joanna Rorbach; Abdul Aziz Yusoff; Helen Tuppen; Dyg P Abg-Kamaludin; Zofia M A Chrzanowska-Lightowlers; Robert W Taylor; Douglass M Turnbull; Robert McFarland; Robert N Lightowlers
Overexpression of human mitochondrial valyl tRNA synthetase can partially restore levels of cognate mt-tRNAVal carrying the pathogenic C25U mutation.
Nucleic acids research 2008;36(9):3065-74.
2007: Joanna Rorbach; R Soleimanpour-Lichaei; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
How do mammalian mitochondria synthesize proteins?
Biochemical Society transactions 2007;35(Pt 5):1290-1.
2007: Hamid Reza Soleimanpour-Lichaei; Inge Kühl; Mauricette Gaisne; Joao Passos; Mateusz Wydro; Joanna Rorbach; Richard Temperley; Nathalie Bonnefoy; Warren Tate; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
mtRF1a is a human mitochondrial translation release factor decoding the major termination codons UAA and UAG.
Molecular cell 2007;27(5):745-57.
2006: Ian Mickleburgh; Hervé Chabanon; David Nury; Kunbo Fan; Brian Burtle; Zofia M A Chrzanowska-Lightowlers; John Hesketh
Elongation factor 1alpha binds to the region of the metallothionein-1 mRNA implicated in perinuclear localization--importance of an internal stem-loop.
RNA (New York, N.Y.) 2006;12(7):1397-407.
2006: Emma L Blakely; Katherine J Rennie; Linda Jones; Matthias Elstner; Zofia M A Chrzanowska-Lightowlers; Christopher B White; Julian P H Shield; Daniela T Pilz; Douglass M Turnbull; Joanna Poulton; Robert W Taylor
Sporadic intragenic inversion of the mitochondrial DNA MTND1 gene causing fatal infantile lactic acidosis.
Pediatric research 2006;59(3):440-4.
2006: Deborah Pye; Dimitra S Kyriakouli; Geoffrey A Taylor; Riem Johnson; Matthias Elstner; Brigitte Meunier; Zofia M A Chrzanowska-Lightowlers; Robert W Taylor; Douglass M Turnbull; Robert N Lightowlers
Production of transmitochondrial cybrids containing naturally occurring pathogenic mtDNA variants.
Nucleic acids research 2006;34(13):e95.
2005: Ian Mickleburgh; Brian Burtle; Hanne Hollås; Gill Campbell; Zofia M A Chrzanowska-Lightowlers; Anni Vedeler; John Hesketh
Annexin A2 binds to the localization signal in the 3' untranslated region of c-myc mRNA.
The FEBS journal 2005;272(2):413-21.
2005: Ruth Seal; Richard J Temperley; Jeffrey Wilusz; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
Serum-deprivation stimulates cap-binding by PARN at the expense of eIF4E, consistent with the observed decrease in mRNA stability.
Nucleic acids research 2005;33(1):376-87.
2004: Ian Mickleburgh; Brian Burtle; David Nury; Hervé Chabanon; Zofia M A Chrzanowska-Lightowlers; John Hesketh
Isolation and identification of a protein binding to the localization element of Metallothionein-1 mRNA.
Biochemical Society transactions 2004;32(Pt 5):705-6.
2004: Olga Kolesnikova; Nina Entelis; Clarisse Jacquin-Becker; Francine Goltzene; Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers; Robert P Martin; Ivan Tarassov
Nuclear DNA-encoded tRNAs targeted into mitochondria can rescue a mitochondrial DNA mutation associated with the MERRF syndrome in cultured human cells.
Human molecular genetics 2004;13(20):2519-34.
2004: Seyed Bidooki; Margaret J Jackson; Margaret A Johnson; Zofia M A Chrzanowska-Lightowlers; Robert W Taylor; Graham Venables; Robert N Lightowlers; Douglass M Turnbull; Laurence A Bindoff
Sporadic mitochondrial myopathy due to a new mutation in the mitochondrial tRNASer(UCN) gene.
Neuromuscular disorders : NMD 2004;14(7):417-20.
2004: Dominique Gagliardi; Piotr P Stepien; Richard J Temperley; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
Messenger RNA stability in mitochondria: different means to an end.
Trends in genetics : TIG 2004;20(6):260-7.
2004: Katharina Maniura-Weber; Robert W Taylor; Margaret A Johnson; Zofia M A Chrzanowska-Lightowlers; Andrew A M Morris; Charles P J Charlton; Douglass M Turnbull; Laurence A Bindoff
A novel point mutation in the mitochondrial tRNA(Trp) gene produces a neurogastrointestinal syndrome.
European journal of human genetics : EJHG 2004;12(6):509-12.
2004: Zofia M A Chrzanowska-Lightowlers; Richard J Temperley; Paul M Smith; Sara H Seneca; Robert N Lightowlers
Functional polypeptides can be synthesized from human mitochondrial transcripts lacking termination codons.
The Biochemical journal 2004;377(Pt 3):725-31.
2003: Richard J Temperley; Sara H Seneca; Katarzyna Tonska; Ewa Bartnik; Laurence A Bindoff; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
Investigation of a pathogenic mtDNA microdeletion reveals a translation-dependent deadenylation decay pathway in human mitochondria.
Human molecular genetics 2003;12(18):2341-8.
2003: Marcus Deschauer; Zofia M A Chrzanowska-Lightowlers; Eckhard Biekmann; Morteza Pourfarzam; Robert W Taylor; Douglass M Turnbull; Stephan Zierz
A splice junction mutation in muscle carnitine palmitoyltransferase II deficiency.
Molecular genetics and metabolism 2003;79(2):124-8.
2003: Pamela R Kearns; Zofia M A Chrzanowska-Lightowlers; Rob Pieters; Anjo J P Veerman; Andrew G Hall
Mu class glutathione S-transferase mRNA isoform expression in acute lymphoblastic leukaemia.
British journal of haematology 2003;120(1):80-8.
2002: Alexei von Delwig; Jeong J Lee; Zofia M A Chrzanowska-Lightowlers; John Robinson
TGF-beta1 and IFN-gamma cross-regulate antigen presentation to CD4 T cells by macrophages.
Journal of leukocyte biology 2002;72(1):163-6.
2002: Fiona M Menzies; Mark Cookson; Robert W Taylor; Douglass M Turnbull; Zofia M A Chrzanowska-Lightowlers; Lichun Dong; Denise A Figlewicz; Pamela J Shaw
Mitochondrial dysfunction in a cell culture model of familial amyotrophic lateral sclerosis.
Brain : a journal of neurology 2002;125(Pt 7):1522-33.
2002: Hoda K Shamloula; Mkajuma P Mbogho; Angel C Pimentel; Zofia M A Chrzanowska-Lightowlers; Vanneta Hyatt; Hideyuki Okano; Tadmiri R Venkatesh
rugose (rg), a Drosophila A kinase anchor protein, is required for retinal pattern formation and interacts genetically with multiple signaling pathways.
Genetics 2002;161(2):693-710.
2001: S S Awad; Robert N Lightowlers; Carol Young; Zofia M A Chrzanowska-Lightowlers; T Lomo; Clarke Slater
Sodium channel mRNAs at the neuromuscular junction: distinct patterns of accumulation and effects of muscle activity.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2001;21(21):8456-63.
2001: A McGregor; Richard J Temperley; Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers
Absence of expression from RNA internalised into electroporated mammalian mitochondria.
Molecular genetics and genomics : MGG 2001;265(4):721-9.
2001: Simon P Selwood; A McGregor; Robert N Lightowlers; Zofia M A Chrzanowska-Lightowlers
Inhibition of mitochondrial protein synthesis promotes autonomous regulation of mtDNA expression and generation of a new mitochondrial RNA species.
FEBS letters 2001;494(3):186-91.
2001: Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers
Fending off decay: a combinatorial approach in intact cells for identifying mRNA stability elements.
RNA (New York, N.Y.) 2001;7(3):435-44.
2000: Frances Gays; M Unnikrishnan; S Shrestha; Karen P Fraser; A R Brown; C M Tristram; Zofia M A Chrzanowska-Lightowlers; Colin G Brooks
The mouse tumor cell lines EL4 and RMA display mosaic expression of NK-related and certain other surface molecules and appear to have a common origin.
Journal of immunology (Baltimore, Md. : 1950) 2000;164(10):5094-102.
2000: Simon P Selwood; Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers
Does the mitochondrial transcription-termination complex play an essential role in controlling differential transcription of the mitochondrial DNA?
Biochemical Society transactions 2000;28(2):154-9.
1999: Jennifer A Toomey; M Salcedo; LA Cotterill; Maggie Millrain; Zofia M A Chrzanowska-Lightowlers; J Lawry; Karen P Fraser; Frances Gays; John Robinson; S Shrestha; PJ Dyson; Colin G Brooks
Stochastic acquisition of Qa1 receptors during the development of fetal NK cells in vitro accounts in part but not in whole for the ability of these cells to distinguish between class I-sufficient and class I-deficient targets.
Journal of immunology (Baltimore, Md. : 1950) 1999;163(6):3176-84.
1999: Kim M Clark; Robert W Taylor; Margaret A Johnson; Patrick F Chinnery; Zofia M A Chrzanowska-Lightowlers; Richard M Andrews; Isabelle P Nelson; Nicholas W Wood; P J Lamont; Michael G Hanna; Robert N Lightowlers; Douglass M Turnbull
An mtDNA mutation in the initiation codon of the cytochrome C oxidase subunit II gene results in lower levels of the protein and a mitochondrial encephalomyopathy.
American journal of human genetics 1999;64(5):1330-9.
1999: Zofia M A Chrzanowska-Lightowlers; Richard J Temperley; A McGregor; Laurence A Bindoff; Robert N Lightowlers
Conversion of a reporter gene for mitochondrial gene expression using iterative mega-prime PCR.
Gene 1999;230(2):241-7.
1998: Jennifer A Toomey; S Shrestha; S A de la Rue; Frances Gays; John Robinson; Zofia M A Chrzanowska-Lightowlers; Colin G Brooks
MHC class I expression protects target cells from lysis by Ly49-deficient fetal NK cells.
European journal of immunology 1998;28(1):47-56.
1997: T Preiss; Zofia M A Chrzanowska-Lightowlers; R N Lightowlers
Glutamate dehydrogenase: an organelle-specific mRNA-binding protein.
Trends in biochemical sciences 1997;22(8):290.
1997: S K Bidooki; Margaret A Johnson; Zofia M A Chrzanowska-Lightowlers; Laurence A Bindoff; Robert N Lightowlers
Intracellular mitochondrial triplasmy in a patient with two heteroplasmic base changes.
American journal of human genetics 1997;60(6):1430-8.
1996: Robert N Lightowlers; A E Sang; T Preiss; Zofia M A Chrzanowska-Lightowlers
Targeting proteins to mitochondria: is there a role for mRNA localization?
Biochemical Society transactions 1996;24(2):527-31.
1995: T Preiss; A E Sang; Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers
The mRNA-binding protein COLBP is glutamate dehydrogenase.
FEBS letters 1995;367(3):291-6.
1995: Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers; Douglass M Turnbull
Gene therapy for mitochondrial DNA defects: is it possible?
Gene therapy 1995;2(5):311-6.
1994: Zofia M A Chrzanowska-Lightowlers; T Preiss; Robert N Lightowlers
Inhibition of mitochondrial protein synthesis promotes increased stability of nuclear-encoded respiratory gene transcripts.
The Journal of biological chemistry 1994;269(44):27322-8.
1994: T Preiss; Zofia M A Chrzanowska-Lightowlers; Robert N Lightowlers
The tissue-specific RNA-binding protein COLBP is differentially regulated during myogenesis.
Biochimica et biophysica acta 1994;1221(3):286-9.
1993: Zofia M A Chrzanowska-Lightowlers; Douglass M Turnbull; Laurence A Bindoff; Robert N Lightowlers
An antisense oligodeoxynucleotide approach to investigate the function of the nuclear-encoded subunits of human cytochrome c oxidase.
Biochemical and biophysical research communications 1993;196(1):328-35.
1993: Zofia M A Chrzanowska-Lightowlers; Douglass M Turnbull; Robert N Lightowlers
A microtiter plate assay for cytochrome c oxidase in permeabilized whole cells.
Analytical biochemistry 1993;214(1):45-9.