Early defects in human T-cell development severely affect distribution and maturation of thymic stromal cells: possible implications for the pathophysiology of Omenn syndrome
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Expression of the autoimmune regulator gene and its relevance to the mechanisms of central and peripheral toleranceRegulatory T-Cell Development in the Human ThymusThe Thymic Orchestration Involving Aire, miRNAs, and Cell-Cell Interactions during the Induction of Central ToleranceT-regulatory cells in primary immune deficienciesAdenosine Deaminase Deficiency - More Than Just an ImmunodeficiencyGenetics of SCIDHuman RAG mutations: biochemistry and clinical implicationsAutoimmunity in primary immune deficiency: taking lessons from our patients.Short-term inhibition of p53 combined with keratinocyte growth factor improves thymic epithelial cell recovery and enhances T-cell reconstitution after murine bone marrow transplantationHuman peripheral lymphoid tissues contain autoimmune regulator-expressing dendritic cellsHomozygous DNA ligase IV R278H mutation in mice leads to leaky SCID and represents a model for human LIG4 syndromeRag defects and thymic stroma: lessons from animal models.Homeostatic expansion of autoreactive immunoglobulin-secreting cells in the Rag2 mouse model of Omenn syndromeExpansion of immunoglobulin-secreting cells and defects in B cell tolerance in Rag-dependent immunodeficiency.Engineering the human thymic microenvironment to support thymopoiesis in vivo.Hypomorphic Rag mutations can cause destructive midline granulomatous disease.CHARGE syndrome: a review of the immunological aspects.Reduced thymic output, cell cycle abnormalities, and increased apoptosis of T lymphocytes in patients with cartilage-hair hypoplasia.Impaired receptor editing and heterozygous RAG2 mutation in a patient with systemic lupus erythematosus and erosive arthritis.Abnormalities of thymic stroma may contribute to immune dysregulation in murine models of leaky severe combined immunodeficiency.PRKDC mutations associated with immunodeficiency, granuloma, and autoimmune regulator-dependent autoimmunity.Reticular dysgenesis-associated AK2 protects hematopoietic stem and progenitor cell development from oxidative stress.Inborn errors of human JAKs and STATs.Intestinal microbiota sustains inflammation and autoimmunity induced by hypomorphic RAG defects.Human FOXN1-deficiency is associated with αβ double-negative and FoxP3+ T-cell expansions that are distinctly modulated upon thymic transplantationImmune Dysfunction in Children with CHARGE Syndrome: A Cross-Sectional Study.Broad-spectrum antibodies against self-antigens and cytokines in RAG deficiencyPartial defects of T-cell development associated with poor T-cell function.The genetic basis of severe combined immunodeficiency and its variants.Treatment of Pediatric Acute Graft-versus-Host Disease-Lessons from Primary Immunodeficiency?Altered T-cell receptor signaling in the pathogenesis of allergic disease.Immunological aspects of 22q11.2 deletion syndrome.Haematopoietic stem cell transplantation for SCID patients: where do we stand?Tolerance and autoimmunity in primary immunodeficiency disease: a comprehensive review.Regulations of gene expression in medullary thymic epithelial cells required for preventing the onset of autoimmune diseases.Immunodeficiency in DiGeorge Syndrome and Options for Treating Cases with Complete AthymiaTracking migration during human T cell development.Clinical Spectrum of SCID: The Key is in the Thymus?Hypomorphic mutation in the RAG2 gene affects dendritic cell distribution and migration.Anti-CD3ε mAb improves thymic architecture and prevents autoimmune manifestations in a mouse model of Omenn syndrome: therapeutic implications.
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P2860
Early defects in human T-cell development severely affect distribution and maturation of thymic stromal cells: possible implications for the pathophysiology of Omenn syndrome
description
article científic
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article scientifique
@fr
articolo scientifico
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artigo científico
@pt
bilimsel makale
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scientific article published on 04 May 2009
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vedecký článok
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vetenskaplig artikel
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videnskabelig artikel
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vědecký článek
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name
Early defects in human T-cell ...... hophysiology of Omenn syndrome
@en
Early defects in human T-cell ...... ophysiology of Omenn syndrome.
@nl
type
label
Early defects in human T-cell ...... hophysiology of Omenn syndrome
@en
Early defects in human T-cell ...... ophysiology of Omenn syndrome.
@nl
prefLabel
Early defects in human T-cell ...... hophysiology of Omenn syndrome
@en
Early defects in human T-cell ...... ophysiology of Omenn syndrome.
@nl
P2093
P2860
P50
P1433
P1476
Early defects in human T-cell ...... hophysiology of Omenn syndrome
@en
P2093
Andrew Richard Gennery
Chaim M Roifman
Luigi D Notarangelo
Maria Ravanini
P2860
P304
P356
10.1182/BLOOD-2009-03-211029
P407
P577
2009-05-04T00:00:00Z