about
Munc18-2 deficiency causes familial hemophagocytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cellsSevere combined immunodeficiency caused by deficiency in either the delta or the epsilon subunit of CD3Sustained correction of X-linked severe combined immunodeficiency by ex vivo gene therapyEculizumab in severe Shiga-toxin-associated HUSXIAP deficiency in humans causes an X-linked lymphoproliferative syndromeAutoimmune lymphoproliferative syndrome with somatic Fas mutationsAutoimmunity in Wiskott-Aldrich syndrome: risk factors, clinical features, and outcome in a single-center cohort of 55 patients.Hematopoietic stem cell transplantation in hemophagocytic lymphohistiocytosis: a single-center report of 48 patients.A novel immunodeficiency associated with hypomorphic RAG1 mutations and CMV infection.Novel mutations in the RFXANK gene: RFX complex containing in-vitro-generated RFXANK mutant binds the promoter without transactivating MHC II.Multiple intestinal atresia with combined immune deficiency related to TTC7A defect is a multiorgan pathology: study of a French-Canadian-based cohort.Improving immune reconstitution while preventing graft-versus-host disease in allogeneic stem cell transplantation.Fatal Mycobacterium colombiense/cytomegalovirus coinfection associated with acquired immunodeficiency due to autoantibodies against interferon gamma: a case report.Whole-exome sequencing reveals a rapid change in the frequency of rare functional variants in a founding population of humans.Cell-death signaling and human disease.Artemis sheds new light on V(D)J recombination.A hypermorphic IkappaBalpha mutation is associated with autosomal dominant anhidrotic ectodermal dysplasia and T cell immunodeficiency.Severe combined immunodeficiency. A model disease for molecular immunology and therapy.CD3 deficiencies.Low penetrance, broad resistance, and favorable outcome of interleukin 12 receptor beta1 deficiency: medical and immunological implications.Implication of different effector mechanisms by cord blood-derived and peripheral blood-derived cytokine-induced killer cells to kill precursor B acute lymphoblastic leukemia cell lines.Cord blood-derived and peripheral blood-derived cytokine-induced killer cells are sensitive to Fas-mediated apoptosis.Cord-blood-derived mesenchymal stromal cells downmodulate CD4+ T-cell activation by inducing IL-10-producing Th1 cells.The VP6 protein of rotavirus interacts with a large fraction of human naive B cells via surface immunoglobulins.Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency).Severe and progressive encephalitis as a presenting manifestation of a novel missense perforin mutation and impaired cytolytic activity.Mechanisms of CD47-induced caspase-independent cell death in normal and leukemic cells: link between phosphatidylserine exposure and cytoskeleton organization.Human mesenchymal stromal cell-secreted lactate induces M2-macrophage differentiation by metabolic reprogramming.T cell-dependent activation of dendritic cells requires IL-12 and IFN-gamma signaling in T cells.High prevalence of primary immune deficiencies in children with autoimmune disorders.T-cell immune constitution after peripheral blood mononuclear cell transplantation in complete DiGeorge syndrome.Ceftazidime-induced drug reaction with eosinophilia and systemic symptoms (DRESS) complicated by hemophagocytic lymphohistiocytosis.Renal granuloma and immunoglobulin M-complex glomerulonephritis: a case of common variable immunodeficiency?Expression of Granzyme B in viral hepatitis in patients with ALPS.Ca2+ ionophores trigger membrane remodeling without a need for store-operated Ca2+ entry.Reconstitution of protective immune responses against cytomegalovirus and varicella zoster virus does not require disease development in pediatric recipients of umbilical cord blood transplantation.A serious adverse event after successful gene therapy for X-linked severe combined immunodeficiency.Hematopoietic engraftment of XLA bone marrow CD34(+) cells in NOG/SCID mice.
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P50
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onderzoeker
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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Françoise Le Deist
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P106
P21
P31
P569
2000-01-01T00:00:00Z