Reduced central tolerance in Omenn syndrome leads to immature self-reactive oligoclonal T cells

Background Omenn syndrome (OS) is characterized by a peculiar severe T-cell immune deficiency associated with autoimmunelike manifestations. Dysregulations of the central and peripheral immune tolerance, mediated by the protein autoimmune regulator (AIRE) and regulatory T cells, respectively, were p...

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Veröffentlicht in:	Journal of allergy and clinical immunology 2009-10, Vol.124 (4), p.793-800
Hauptverfasser:	Somech, Raz, MD, PhD, Simon, Amos J., PhD, Lev, Atar, MSc, Dalal, Ilan, MD, Spirer, Zvi, MD, Goldstein, Itamar, MD, Nagar, Meital, MSc, Amariglio, Ninette, PhD, Rechavi, Gideon, MD, PhD, Roifman, Chaim M., MD
Format:	Artikel
Sprache:	eng
Schlagworte:	AIRE AIRE Protein Allergy and Immunology autoimmunity Autoimmunity - genetics Autoimmunity - immunology Biological and medical sciences CD4-Positive T-Lymphocytes - immunology CD4-Positive T-Lymphocytes - metabolism Clone Cells - immunology Clone Cells - metabolism Cytokines - immunology Cytokines - metabolism Deoxyribonucleic acid DNA Down-Regulation - genetics Down-Regulation - immunology Female Forkhead Transcription Factors - immunology Forkhead Transcription Factors - metabolism Foxp3 Fundamental and applied biological sciences. Psychology Fundamental immunology Gene Expression Gene Rearrangement, T-Lymphocyte Humans Immune Tolerance Immunodeficiencies Immunodeficiencies. Immunoglobulinopathies immunodeficiency Immunopathology Lymphocytes Male Medical sciences oligoclones Omenn syndrome Pathogenesis Patients Receptors, Antigen, T-Cell - genetics Receptors, Antigen, T-Cell - immunology Receptors, Antigen, T-Cell - metabolism Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis SCID Severe Combined Immunodeficiency - genetics Severe Combined Immunodeficiency - immunology Severe Combined Immunodeficiency - metabolism T cell receptors T-Lymphocytes, Regulatory - immunology T-Lymphocytes, Regulatory - metabolism tolerance Transcription Factors - immunology Transcription Factors - metabolism TREC Treg Up-Regulation - genetics Up-Regulation - immunology
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