title: Analysis of the peripheral T-cell compartment in the MHC class II deficiency syndrome.
abstract: To analyse the impact of lack of MHC class II expression on the composition of the peripheral T-cell compartment in man, the expression characteristics of several membrane antigens were examined on peripheral blood lymphocytes (PBL) and cultured T cells derived from an MHC-class-II-deficient patient. No MHC class II expression could be detected on either PBL or activated T cells. Moreover, the expression of MHC class I was reduced both on PBL and in vitro activated T cells compared to the healthy control. However, the reduced expression of CD26 observed on the PBL of the patient was restored after in vitro expansion. Despite the presumably class-II-deficient thymic environment, a distinct but reduced single CD4+ T-cell population was observed in the PBL of the patient. After in vitro expansion, the percentage of CD4+ cells dropped even further, most likely due to a proliferative disadvantage, compared to the single CD8+ T-cell population. However, proliferation analysis showed that T-cell activation via the TcR/CD3 pathway is not affected by the MHC class II deficiency.
authors.affiliation: Department of Immunohematology, University Hospital, Leiden, The Netherlands.
authors.forename: M
authors.initials: M
authors.lastname: Lambert
authors.forename: M
authors.initials: M
authors.lastname: van Eggermond
authors.forename: M
authors.initials: M
authors.lastname: Andrien
authors.forename: F
authors.initials: F
authors.lastname: Mascart
authors.forename: E
authors.initials: E
authors.lastname: Vamos
authors.forename: E
authors.initials: E
authors.lastname: Dupont
authors.forename: P
authors.initials: P
authors.lastname: van den Elsen
mesh_terms: D015703:Antigens, CD / Q000276:immunology*; D015152:Blotting, Northern; D002460:Cell Line; D005434:Flow Cytometry; D005802:Genes, MHC Class II; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D008213:Lymphocyte Activation / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology*
chemical_list: D015703:Antigens, CD