title: Transcriptional control of MHC genes and T cell development in MHC class II deficiency.
abstract: MHC class II deficiency has proven to be an excellent model to study transcription regulation of MHC genes and T cell development. Cell lines established from MHC class II deficient patients have been of great value for the identification of proteins necessary for MHC expression and their study has resulted in the identification of a common regulatory pathway for MHC class II and class I genes. The lack of MHC class II expression was found to have a profound effect on the development of the CD4+ T cell lineage, in particular on the composition of the T cell receptor repertoire, revealing aberrant thymic selection processes in these patients. Here, we will discuss several aspects of the transcriptional regulation of MHC genes and the impact of deficient MHC class II expression on T cell development.
authors.affiliation: Department of Immunohematology and Blood Bank, Leiden University Medical Center, The Netherlands.
authors.forename: B
authors.initials: B
authors.lastname: Godthelp
authors.forename: M C
authors.initials: MC
authors.lastname: Van Eggermond
authors.forename: S J
authors.initials: SJ
authors.lastname: Gobin
authors.forename: P J
authors.initials: PJ
authors.lastname: Van den Elsen
mesh_terms: D015496:CD4-Positive T-Lymphocytes / Q000276:immunology; D002454:Cell Differentiation / Q000276:immunology; D005805:Genes, MHC Class I; D005802:Genes, MHC Class II; D000949:Histocompatibility Antigens Class II / Q000378:metabolism; D006801:Humans; D016511:Severe Combined Immunodeficiency / Q000235:genetics* / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology*; D014158:Transcription, Genetic
chemical_list: D000949:Histocompatibility Antigens Class II