Abstract
The CD40 ligand expressed on activated T cells plays a pivotal role in B cell proliferation and differentiation. Mutations in the CD40 ligand gene, which alter its expression on the surface of activated T cells, are associated with the X-linked form of Hyper-IgM syndrome (XHIM). A rapid and simple, three-color whole blood flow cytometry procedure was developed for maximal expression and detection of the CD40L on the surface of in vitro activated CD4+ T cells. Approximately 90% of in vitro activated CD4+ T cells obtained from healthy controls expressed the CD40L compared to only 5% of in vitro activated CD4+ T cells obtained from the XHIM patients. The CD40L was expressed on approximately 50% of the in vitro activated CD4+ T cells obtained from the mothers of XHIM patients, consistent with a diagnosis of their carrier status. This is the first report of a whole blood procedure adapted for routine clinical use which is able to detect abnormal CD40L expression in XHIM patients and carriers.
Original language | English (US) |
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Pages (from-to) | 172-181 |
Number of pages | 10 |
Journal | Clinical Immunology and Immunopathology |
Volume | 85 |
Issue number | 2 |
DOIs | |
State | Published - Jan 1 1997 |
ASJC Scopus subject areas
- Immunology and Allergy
- Pathology and Forensic Medicine
- Immunology