Publications by authors named "Claire Booth"

42Publications

Gene therapy for primary immunodeficiencies: up-to-date.

Expert Opin Biol Ther 2020 Oct 29:1-10. Epub 2020 Oct 29.

Department of Infection, Immunity and Inflammation, UCL Great Ormond Street Institute of Child Health , London, United Kingdom.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1080/14712598.2021.1837108DOI Listing
October 2020

Successful Auxiliary Liver Transplant Followed by Hematopoietic Stem Cell Transplantation in X-Linked Lymphoproliferative Disease Type 1.

Liver Transpl 2020 Sep 19. Epub 2020 Sep 19.

Paediatric Centre for Hepatology, Gastroenterology and Nutrition, King's College Hospital, London, United Kingdom.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1002/lt.25898DOI Listing
September 2020

Chest Radiographs for Distinguishing ADA-SCID from Other Forms of SCID.

J Clin Immunol 2020 02 19;40(2):259-266. Epub 2019 Dec 19.

Department of Paediatric Radiology, Great Ormond Street Hospital for Children, Great Ormond Street, London, WC1N 3JH, UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10875-019-00733-1DOI Listing
February 2020

Janus kinase inhibition for autoinflammation in patients with DNASE2 deficiency.

J Allergy Clin Immunol 2020 02 24;145(2):701-705.e8. Epub 2019 Nov 24.

University College London Great Ormond Street Institute of Child Health, London, United Kingdom; Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaci.2019.11.020DOI Listing
February 2020

Haematopoietic Stem Cell Transplantation for Primary Haemophagocytic Lymphohistiocytosis.

Front Pediatr 2019 25;7:435. Epub 2019 Oct 25.

Department of Paediatric Immunology, Great Ormond Street Hospital, London, United Kingdom.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.3389/fped.2019.00435DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6823612PMC
October 2019

Gene therapy for primary immunodeficiency.

Hum Mol Genet 2019 10;28(R1):R15-R23

Molecular and Cellular Immunology Section, UCL Great Ormond Street Institute of Child Health, London, UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1093/hmg/ddz170DOI Listing
October 2019

Different Phenotypic Presentations of X-Linked Lymphoproliferative Disease in Siblings with Identical Mutations.

J Clin Immunol 2019 07 29;39(5):523-526. Epub 2019 May 29.

Department of Pediatric Immunology, Great Ormond Street Hospital, London, WC1N 3JH, UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1007/s10875-019-00649-wDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7086673PMC
July 2019

Outcomes and Treatment Strategies for Autoimmunity and Hyperinflammation in Patients with RAG Deficiency.

J Allergy Clin Immunol Pract 2019 Jul - Aug;7(6):1970-1985.e4. Epub 2019 Mar 12.

University of South Florida and Johns Hopkins All Children's Hospital, Saint Petersburg, Fla; Division of Allergy and Immunology, Massachusetts General Hospital for Children, Boston, Mass. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaip.2019.02.038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6612449PMC
September 2020

Hematopoietic stem cell transplantation for CD40 ligand deficiency: Results from an EBMT/ESID-IEWP-SCETIDE-PIDTC study.

J Allergy Clin Immunol 2019 06 17;143(6):2238-2253. Epub 2019 Jan 17.

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

View Article

Download full-text PDF

Source
https://linkinghub.elsevier.com/retrieve/pii/S00916749193003
Publisher Site
http://dx.doi.org/10.1016/j.jaci.2018.12.1010DOI Listing
June 2019

A Spectrum of Genetic Variants Contributes to Immune Defects and Pathogenesis of Inflammatory Bowel Diseases.

Gastroenterology 2018 06 5;154(8):2022-2024. Epub 2018 May 5.

Molecular and Cellular Immunology Section, UCL GOS Institute of Child Health and Department of Paediatric Immunology, Great Ormond Street Hospital, London, UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1053/j.gastro.2018.05.001DOI Listing
June 2018

Transfer of gene-corrected T cells corrects humoral and cytotoxic defects in patients with X-linked lymphoproliferative disease.

J Allergy Clin Immunol 2018 07 27;142(1):235-245.e6. Epub 2018 Apr 27.

Molecular and Cellular Immunology Section, UCL GOS Institute of Child Health, London, United Kingdom; Department of Paediatric Immunology, Great Ormond Street Hospital NHS Trust, London, United Kingdom. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaci.2018.02.053DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6034012PMC
July 2018

X-Linked Lymphoproliferative Disease Type 1: A Clinical and Molecular Perspective.

Front Immunol 2018 4;9:666. Epub 2018 Apr 4.

National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, United States.

View Article

Download full-text PDF

Source
http://journal.frontiersin.org/article/10.3389/fimmu.2018.00
Publisher Site
http://dx.doi.org/10.3389/fimmu.2018.00666DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5893764PMC
June 2019

T-cell gene therapy for perforin deficiency corrects cytotoxicity defects and prevents hemophagocytic lymphohistiocytosis manifestations.

J Allergy Clin Immunol 2018 09 31;142(3):904-913.e3. Epub 2018 Jan 31.

Infection, Immunity, Inflammation, Molecular and Cellular Immunology Section, UCL Great Ormond Street Institute of Child Health, London, United Kingdom. Electronic address:

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaci.2017.11.050DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6127027PMC
September 2018

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.

View Article

Download full-text PDF

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

View Article

Download full-text PDF

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

Hematopoietic stem cell transplant in patients with activated PI3K delta syndrome.

J Allergy Clin Immunol 2017 03 12;139(3):1046-1049. Epub 2016 Nov 12.

Great North Children's Hospital, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, United Kingdom; Primary Immunodeficiency Group, Institute of Cellular Medicine, Newcastle upon Tyne University, Newcastle upon Tyne, United Kingdom.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.jaci.2016.09.040DOI Listing
March 2017

Severe Epstein-Barr virus infection in primary immunodeficiency and the normal host.

Br J Haematol 2016 Nov 17;175(4):559-576. Epub 2016 Oct 17.

Department of Immunology, Great Ormond Street Hospital, London, UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/bjh.14339DOI Listing
November 2016

Stepwise phosphorylation of p65 promotes NF-κB activation and NK cell responses during target cell recognition.

Nat Commun 2016 05 25;7:11686. Epub 2016 May 25.

Department of Biomedical Sciences, University of Ulsan College of Medicine, 86 Asanbyeongwon-Gil, Seoul 138-735, Korea.

View Article

Download full-text PDF

Source
http://www.nature.com/doifinder/10.1038/ncomms11686
Publisher Site
http://dx.doi.org/10.1038/ncomms11686DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4894962PMC
May 2016

Treating Immunodeficiency through HSC Gene Therapy.

Trends Mol Med 2016 Apr 15;22(4):317-327. Epub 2016 Mar 15.

Molecular and Cellular Immunology Section, UCL Institute of Child Health, London, UK; Department of Paediatric Immunology, Great Ormond Street Hospital, London, UK. Electronic address:

View Article

Download full-text PDF

Source
http://www.cell.com/trends/molecular-medicine/pdf/S1471-4914
Web Search
http://linkinghub.elsevier.com/retrieve/pii/S147149141600032
Publisher Site
http://dx.doi.org/10.1016/j.molmed.2016.02.002DOI Listing
April 2016

Stem cell transplantation for the treatment of immunodeficiency in children: current status and hopes for the future.

Expert Rev Clin Immunol 2016 Jul 4;12(7):713-23. Epub 2016 Mar 4.

b Department of Bone Marrow Transplantation , Great Ormond Street Hospital , London , UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1586/1744666X.2016.1150177DOI Listing
July 2016

Lentivirus technologies for modulation of the immune system.

Curr Opin Pharmacol 2015 Oct 10;24:119-27. Epub 2015 Sep 10.

Molecular and Cellular Immunology, Institute of Child Health, University College London, London WC1N 1EH, UK; Department of Paediatric Immunology, Great Ormond Street Hospital NHS Foundation Trust, London WC1N 3JH, UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.coph.2015.08.007DOI Listing
October 2015

Gene therapy for haemophagocytic lymphohistiocytosis.

Curr Gene Ther 2014 ;14(6):437-46

Molecular Immunology Unit, UCL Institute of Child Health, 30, Guilford Street, London WC1N 1EH.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.2174/1566523214666140918112113DOI Listing
July 2015

Stem cell transplantation for primary immune deficiency.

Curr Opin Hematol 2013 Nov;20(6):501-8

aDepartment of Paediatric Immunology, Great Ormond Street Hospital NHS Foundation Trust bMolecular Immunology Unit, Institute of Child Health, University College London cDepartment of Bone Marrow Transplantation, Great Ormond Street Hospital NHS Foundation Trust, London, UK.

View Article

Download full-text PDF

Source
http://content.wkhealth.com/linkback/openurl?sid=WKPTLP:land
Publisher Site
http://dx.doi.org/10.1097/MOH.0b013e328365a13bDOI Listing
November 2013

The current role of T cell depletion in paediatric stem cell transplantation.

Br J Haematol 2013 Jul 30;162(2):177-90. Epub 2013 May 30.

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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1111/bjh.12400DOI Listing
July 2013

SAP gene transfer restores cellular and humoral immune function in a murine model of X-linked lymphoproliferative disease.

Blood 2013 Feb 5;121(7):1073-6. Epub 2012 Dec 5.

Centre for Immunodeficiency, Molecular Immunology Unit, UCL Institute of Child Health, London, United Kingdom.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1182/blood-2012-07-445858DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3779401PMC
February 2013

Gene therapy for primary immunodeficiency.

Curr Opin Pediatr 2011 Dec;23(6):659-66

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

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1097/MOP.0b013e32834cd67aDOI Listing
December 2011

Pegademase bovine (PEG-ADA) for the treatment of infants and children with severe combined immunodeficiency (SCID).

Biologics 2009 13;3:349-58. Epub 2009 Jul 13.

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

View Article

Download full-text PDF

Source
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2726071PMC
February 2010

Management options for adenosine deaminase deficiency; proceedings of the EBMT satellite workshop (Hamburg, March 2006).

Clin Immunol 2007 May 14;123(2):139-47. Epub 2007 Feb 14.

Molecular Immunology Unit, Institute of Child Health, University College London, and Department of Clinical Immunology, Great Ormond Street Hospital NHS Trust, London WC1N 3JH, UK.

View Article

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clim.2006.12.009DOI Listing
May 2007