THE BARE LYMPHOCYTE SYNDROME AND THE REGULATION OF MHC EXPRESSION

The bare lymphocyte syndrome (BLS) is a hereditary immunodeficiency resulting from the absence of major istocompatibility complex class II (MHCII) expression. Considering the central role of MHCII molecules in the development and activation of CD4 + T cells, it is not surprising that the immune syst...

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Veröffentlicht in:	Annual review of immunology 2001-01, Vol.19 (1), p.331-373
Hauptverfasser:	Reith, Walter, Mach, Bernard
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals bare lymphocyte syndrome CD4 antigen CIITA CIITA protein Disease Susceptibility DNA-Binding Proteins - deficiency DNA-Binding Proteins - genetics DNA-Binding Proteins - physiology Gene Expression Regulation - immunology Genes, MHC Class II Genes, Recessive Genetic Complementation Test Genetic Heterogeneity histocompatibility antigen HLA Histocompatibility Antigens Class II - biosynthesis Histocompatibility Antigens Class II - genetics Histocompatibility Antigens Class II - immunology Humans Macromolecular Substances MHC class II deficiency Mice Mice, Knockout Models, Animal Models, Immunological Nuclear Proteins primary immunodeficiency disease Promoter Regions, Genetic Protein Subunits Regulatory Factor X Transcription Factors RFX RFX5 protein RFXANK protein RFXAP protein Severe Combined Immunodeficiency - genetics Severe Combined Immunodeficiency - immunology Trans-Activators - deficiency Trans-Activators - genetics Trans-Activators - physiology Transcription Factors - deficiency Transcription Factors - genetics Transcription Factors - physiology transcription regulation Transcriptional Activation
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