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Residual MHC class II expression on mature dendritic cells and activated B cells in RFX5-deficient mice

Clausen, B. E.
Schwenk, F.
Rajewsky, K.
Forster, I.
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 AnimalsB-Lymphocytes/ immunologyDNA-Binding Proteins/ geneticsDendritic Cells/ immunologyGene Expression Regulation, DevelopmentalGene TargetingGenes, MHC Class IIGenetic VectorsH-2 Antigens/biosynthesis/geneticsHistocompatibility Antigens Class II/ biosynthesisImmunologic Deficiency Syndromes/geneticsInterferon-gamma/pharmacologyLiver/immunologyLymphocyte ActivationMacrophages, Peritoneal/drug effects/immunologyMiceMice, KnockoutMutationRNA, Messenger/analysisSpleen/immunologyThymus Gland/immunology
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PMID: 9491996
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