T-Cell Repertoire Development in MHC Class II Deficient Humans

  • Marja van Eggermond
  • Marijke Lambert
  • Françoise Mascart
  • Etienne Dupont
  • Peter van den Elsen
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 329)


We have investigated the impact of MHC class II deficiency on the development of the peripheral T-cell repertoire. During T-cell development, interactions between T-cell receptor (TcR) and MHC class I and class II molecules play a pivotal role in the positive and negative selection events that occur within the thymic micro-environment1,2, 3, 4,5. These MHC dependent selection processes influence in greater part the composition of the peripheral T-cell compartment, where only T-cells with moderate affinity for “self” MHC class I or class II molecules can be found6. Various studies in the mouse have shown that interference in these TcR/MHC interactions has a dramatic effect on the composition of the peripheral T-cell compartment with respect to development of T-cells co-expressing the accessory molecules CD4 or CD8 respectively.7,8,9,10


Gene Segment Segment Usage Deficient Environment Deficient Human Bare Lymphocyte Syndrome 
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Copyright information

© Springer Science+Business Media New York 1993

Authors and Affiliations

  • Marja van Eggermond
    • 1
  • Marijke Lambert
    • 1
  • Françoise Mascart
    • 2
  • Etienne Dupont
    • 2
  • Peter van den Elsen
    • 1
  1. 1.Department of Immunohaematology and BloodbankUniversity Hospital LeidenRC LeidenThe Netherlands
  2. 2.Department of Immunology Haematology and TransfusionHôpital ErasmeBrusselsBelgium

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