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Residual MHC class II expression on mature dendritic cells and activated B cells in RFX5-deficient mice
|Published in||Immunity. 1998, vol. 8, no. 2, p. 143-155|
|Abstract||Patients with major histocompatibility complex class II (MHC-II) deficiency are known to carry mutations in either the RFX complex or the trans-activator CIITA. While the pivotal role of CIITA for MHC-II gene transcription is supported by the essential absence of MHC-II molecules in CIITA-deficient mice, we demonstrate here that RFX5-/- mice retain expression of MHC-II in thymic medulla, mature dendritic cells, and activated B cells. Nevertheless, RFX5-/- mice develop a severe immunodeficiency due to the lack of MHC-II in thymic cortex, failure of positive selection of CD4+ T cells, and absence of MHC-II on resting B cells and resident or IFNgamma-activated macrophages. This differential requirement for CIITA and RFX5 in subsets of antigen-presenting cells may be specific for the mouse; it may, however, also exist in humans without having been noticed so far.|
|Keywords||Animals — B-Lymphocytes/ immunology — DNA-Binding Proteins/ genetics — Dendritic Cells/ immunology — Gene Expression Regulation, Developmental — Gene Targeting — Genes, MHC Class II — Genetic Vectors — H-2 Antigens/biosynthesis/genetics — Histocompatibility Antigens Class II/ biosynthesis — Immunologic Deficiency Syndromes/genetics — Interferon-gamma/pharmacology — Liver/immunology — Lymphocyte Activation — Macrophages, Peritoneal/drug effects/immunology — Mice — Mice, Knockout — Mutation — RNA, Messenger/analysis — Spleen/immunology — Thymus Gland/immunology|