Siobhan O Burns

Siobhan O Burns

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Siobhan O Burns

Siobhan O Burns

Publications by authors named "Siobhan O Burns"

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Expanding Clinical Phenotype and Novel Insights into the Pathogenesis of ICOS Deficiency.

J Clin Immunol 2019 Dec 20. Epub 2019 Dec 20.

NIH Research-Leeds Biomedical Research Centre and Leeds Institute of Rheumatic and Musculoskeletal Medicine (LIRMM), Wellcome Trust Brenner Building, St. James's University Hospital, Beckett Street, Leeds, UK.

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http://dx.doi.org/10.1007/s10875-019-00735-zDOI Listing
December 2019

A comprehensive characterization of chronic norovirus infection in immunodeficient hosts.

J Allergy Clin Immunol 2019 Nov 13;144(5):1450-1453. Epub 2019 Aug 13.

Institute of Immunity and Transplantation, University College London, Royal Free Campus, London, United Kingdom; Department of Clinical Immunology, Royal Free London NHS Foundation Trust, London, United Kingdom. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00916749193103
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http://dx.doi.org/10.1016/j.jaci.2019.07.036DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6843911PMC
November 2019

Respiratory Infections and Antibiotic Usage in Common Variable Immunodeficiency.

J Allergy Clin Immunol Pract 2018 Jan - Feb;6(1):159-168.e3. Epub 2017 Jul 19.

Department of Clinical Immunology, Royal Free London NHS Foundation Trust, London, United Kingdom; Institute of Immunity and Transplantation, University College London, London, United Kingdom. Electronic address:

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http://dx.doi.org/10.1016/j.jaip.2017.05.024DOI Listing
October 2019

Disease Evolution and Response to Rapamycin in Activated Phosphoinositide 3-Kinase δ Syndrome: The European Society for Immunodeficiencies-Activated Phosphoinositide 3-Kinase δ Syndrome Registry.

Front Immunol 2018 16;9:543. Epub 2018 Mar 16.

Center for Chronic Immunodeficiency, Medical Center - University of Freiburg, Freiburg, Germany.

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http://journal.frontiersin.org/article/10.3389/fimmu.2018.00
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http://dx.doi.org/10.3389/fimmu.2018.00543DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5863269PMC
May 2019

How I manage patients with Wiskott Aldrich syndrome.

Br J Haematol 2019 May 12;185(4):647-655. Epub 2019 Mar 12.

Department of Immunology, Royal Free London NHS Foundation Trust, London, UK.

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http://dx.doi.org/10.1111/bjh.15831DOI Listing
May 2019

Bleeding and splenectomy in Wiskott-Aldrich syndrome: A single-centre experience.

J Allergy Clin Immunol Pract 2019 Mar 23;7(3):1042-1044.e1. Epub 2018 Jul 23.

Department of Immunology, Royal Free London NHS Foundation Trust, London, United Kingdom; University College London, Institute of Immunity and Transplantation, London, United Kingdom.

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http://dx.doi.org/10.1016/j.jaip.2018.07.009DOI Listing
March 2019

Study of an extended family with CTLA-4 deficiency suggests a CD28/CTLA-4 independent mechanism responsible for differences in disease manifestations and severity.

Clin Immunol 2018 03 3;188:94-102. Epub 2018 Jan 3.

Seccion de Infectología e Inmunopatología, Unidad de Pediatria, Hospital Virgen del Rocío, Sevilla, Instituto de Biomedicina de Sevilla (IBiS), Spain.

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http://dx.doi.org/10.1016/j.clim.2018.01.001DOI Listing
March 2018

Absence of γ-Chain in Keratinocytes Alters Chemokine Secretion, Resulting in Reduced Immune Cell Recruitment.

J Invest Dermatol 2017 10 17;137(10):2120-2130. Epub 2017 Jun 17.

Institute of Immunity and Transplantation, University College London, London, UK; Department of Immunology, Royal Free Hospital Foundation Trust, London, UK. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S0022202X173164
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http://dx.doi.org/10.1016/j.jid.2017.05.024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5896775PMC
October 2017

Corrigendum: Natural Killer Cells from Patients with Recombinase-Activating Gene and Non-Homologous End Joining Gene Defects Comprise a Higher Frequency of CD56 NKG2A Cells, and Yet Display Increased Degranulation and Higher Perforin Content.

Front Immunol 2017 10;8:1244. Epub 2017 Oct 10.

Laboratory of Host Defenses, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States.

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http://journal.frontiersin.org/article/10.3389/fimmu.2017.01
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http://dx.doi.org/10.3389/fimmu.2017.01244DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5641412PMC
October 2017

Inflammatory and autoimmune manifestations in X-linked carriers of chronic granulomatous disease in the United Kingdom.

J Allergy Clin Immunol 2017 08 23;140(2):628-630.e6. Epub 2017 Mar 23.

Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, United Kingdom; Department of Paediatric Immunology, Great North Children's Hospital, Newcastle upon Tyne, United Kingdom.

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http://dx.doi.org/10.1016/j.jaci.2017.02.029DOI Listing
August 2017

Natural Killer Cells from Patients with Recombinase-Activating Gene and Non-Homologous End Joining Gene Defects Comprise a Higher Frequency of CD56 NKG2A Cells, and Yet Display Increased Degranulation and Higher Perforin Content.

Front Immunol 2017 17;8:798. Epub 2017 Jul 17.

Laboratory of Host Defenses, Division of Intramural Research, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, United States.

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http://journal.frontiersin.org/article/10.3389/fimmu.2017.00
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http://dx.doi.org/10.3389/fimmu.2017.00798DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5511964PMC
July 2017

Clinical spectrum and features of activated phosphoinositide 3-kinase δ syndrome: A large patient cohort study.

J Allergy Clin Immunol 2017 02 16;139(2):597-606.e4. Epub 2016 Jul 16.

Department of Paediatric Immunology, Newcastle upon Tyne hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne Hospitals NHS Trust, Newcastle upon Tyne, United Kingdom.

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http://dx.doi.org/10.1016/j.jaci.2016.06.021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5292996PMC
February 2017

Primary immunodeficiencies due to abnormalities of the actin cytoskeleton.

Curr Opin Hematol 2017 01;24(1):16-22

aUniversity College London Institute of Immunity and Transplantation bDepartment of Immunology, Royal Free London NHS Foundation Trust cUniversity College London Institute of Child Health dGreat Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

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http://dx.doi.org/10.1097/MOH.0000000000000296DOI Listing
January 2017

Lentiviral Vector-Mediated Correction of a Mouse Model of Leukocyte Adhesion Deficiency Type I.

Hum Gene Ther 2016 09 5;27(9):668-78. Epub 2016 May 5.

1 Division of Hematopoietic Innovative Therapies, Centro de Investigaciones Energéticas, Medioambientales y Tecnológicas (CIEMAT) , and Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER), Madrid, Spain .

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5035374PMC
http://dx.doi.org/10.1089/hum.2016.016DOI Listing
September 2016

Common variable immunodeficiency and natural killer cell lymphopenia caused by Ets-binding site mutation in the IL-2 receptor γ (IL2RG) gene promoter.

J Allergy Clin Immunol 2016 Mar 31;137(3):940-2.e4. Epub 2015 Oct 31.

Molecular and Cellular Immunology, Institute of Child Health, University College London, London, United Kingdom; Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom.

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http://dx.doi.org/10.1016/j.jaci.2015.08.049DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4774944PMC
March 2016

Immunodeficiency and severe susceptibility to bacterial infection associated with a loss-of-function homozygous mutation of MKL1.

Blood 2015 Sep 29;126(13):1527-35. Epub 2015 Jul 29.

Department of Immunology, Royal Free London NHS Foundation Trust, London, United Kingdom; and University College London Institute of Immunity and Transplantation, London, United Kingdom.

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http://dx.doi.org/10.1182/blood-2014-12-611012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4671330PMC
September 2015

Modelling of human Wiskott-Aldrich syndrome protein mutants in zebrafish larvae using in vivo live imaging.

J Cell Sci 2013 Sep 18;126(Pt 18):4077-84. Epub 2013 Jul 18.

School of Biochemistry, Faculty of Medical and Veterinary Sciences, University of Bristol, University Walk, Bristol, BS8 1TD, UK.

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http://dx.doi.org/10.1242/jcs.128728DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3772384PMC
September 2013

Cytoskeletal remodeling mediated by WASp in dendritic cells is necessary for normal immune synapse formation and T-cell priming.

Blood 2011 Sep 20;118(9):2492-501. Epub 2011 Jun 20.

Molecular Immunology Unit, Centre for Immunodeficiency, University College London (UCL) Institute of Child Health, London, UK.

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http://www.bloodjournal.org/cgi/doi/10.1182/blood-2011-03-34
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http://dx.doi.org/10.1182/blood-2011-03-340265DOI Listing
September 2011

Lentivectors are efficient tools to manipulate the dendritic cell cytoskeleton.

Cytoskeleton (Hoboken) 2011 Aug 29;68(8):434-45. Epub 2011 Jul 29.

Molecular Immunology Unit, Wolfson Centre for Gene Therapy of Childhood Disease, UCL Institute of Child Health, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom.

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http://dx.doi.org/10.1002/cm.20521DOI Listing
August 2011

Recent advances in the understanding of genetic defects of neutrophil number and function.

Br J Haematol 2010 Nov 31;151(4):312-26. Epub 2010 Aug 31.

Molecular Immunology Unit, UCL Institute of Child Health, Great Ormond Street Hospital NHS Trust, London, UK.

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http://dx.doi.org/10.1111/j.1365-2141.2010.08361.xDOI Listing
November 2010

A congenital activating mutant of WASp causes altered plasma membrane topography and adhesion under flow in lymphocytes.

Blood 2010 Jul 30;115(26):5355-65. Epub 2010 Mar 30.

Molecular Immunology Unit, Centre for Immunodeficiency, Institute of Child Health, University College London, London, UK.

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http://dx.doi.org/10.1182/blood-2009-08-236174DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2951698PMC
July 2010

WASP: a key immunological multitasker.

Nat Rev Immunol 2010 Mar;10(3):182-92

Molecular Immunology Unit and Centre for Immunodeficiency, University College London Institute of Child Health, London, UK.

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http://dx.doi.org/10.1038/nri2724DOI Listing
March 2010

Phosphorylation of WASp is a key regulator of activity and stability in vivo.

Proc Natl Acad Sci U S A 2009 Sep 1;106(37):15738-43. Epub 2009 Sep 1.

Molecular Immunology Unit, Wolfson Centre for Gene Therapy of Childhood Disease and Centre for Immunodeficiency, UCL Institute of Child Health, London, WC1N 1EH, United Kingdom.

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http://dx.doi.org/10.1073/pnas.0904346106DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2736139PMC
September 2009