O'Gorman M R, Zaas D, Paniagua M, Corrochano V, Scholl P R, Pachman L M
Children's Memorial Hospital, Northwestern University, Chicago, Illinois 60614, USA.
Clin Immunol Immunopathol. 1997 Nov;85(2):172-81. doi: 10.1006/clin.1997.4422.
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.
活化T细胞上表达的CD40配体在B细胞增殖和分化中起关键作用。CD40配体基因的突变会改变其在活化T细胞表面的表达，与X连锁高IgM综合征（XHIM）有关。我们开发了一种快速简单的三色全血流式细胞术程序，用于体外活化的CD4+T细胞表面CD40L的最大表达和检测。与从XHIM患者获得的体外活化CD4+T细胞中仅5%相比，从健康对照获得的约90%的体外活化CD4+T细胞表达CD40L。从XHIM患者母亲获得的体外活化CD4+T细胞中约50%表达CD40L，这与其携带者状态的诊断一致。这是首次报道适用于常规临床使用的全血程序，该程序能够检测XHIM患者和携带者中异常的CD40L表达。
Zotero 插件