Normal T cell receptor Vβ usage in a primary immunodeficiency associated with HLA class II deficiency

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Abstract
The human Tcell receptor was studied using an anchored‐polymerase chain reaction (A‐PCR) and hybridization with Vβ‐specific oligonucleotide probes, together with the few anti‐Vβ monoclonal antibodies (mAb) available. After confirming the semiquantitative and reproducible nature of the A‐PCR technique, we assessed the complete Vβ repertoire in sorted CD4+ and CD8+ lymphocyte populations from three normal donors. These experiments confirmed the absence of Vβ‐restricted deletions in human peripheral cells, in contrast to several mouse strains. This feature makes it difficult to study negative selection in man, given the apparent absence of an endogenous superantigen corresponding to the Mis system in the mouse. To investigate human Vβ repertoire shaping, we studied VP usage in CD4+ and CD8+ T cells from children with an inherited immunodeficiency characterized by defective expression of human leukocyte antigen class II molecules. An initial study using anti‐Vβ monoclonal antibodies failed to show significant abnormalities in Vβ usage. Four patients analyzed using the A‐PCR method all had a polyclonal Vβ repertoire, suggesting normal positive selection and raising questions as to the importance of VP major histocompatibility complex (MHC) interactions and the role of thymic MHC density in shaping the Vβ repertoire.