Signaling through CD40 rescues IgE but not IgG or IgA secretion in X-linked immunodeficiency with hyper-IgM.

O Saiki; T Tanaka; Y Wada; H Uda; A Inoue; Y Katada; M Izeki; M Iwata; H Nunoi; I Matsuda

doi:10.1172/JCI117692

Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Department of Clinical Investigation, Osaka Minami National Hospital, Japan.

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Published in Volume 95, Issue 2 on February 1, 1995
J Clin Invest. 1995;95(2):510–514. https://doi.org/10.1172/JCI117692.
© 1995 The American Society for Clinical Investigation

Published February 1, 1995 - Version history

The ligand for CD40 (CD40L) is a membrane protein on activated T cells that induces B cell proliferation and differentiation. Several mutations of the CD40L gene were reported responsible for defective class switching of B cells in an X-linked immunodeficiency with hyper IgM (X-HIM). We studied four affected males from three families and found three independent mutations including new mutations of CD40L gene. In every X-HIM patient tested, however, anti-CD40 plus IL-10 did not induce class switching from IgM to IgG or IgA, even in the presence of Staphylococcus aureus Cowan I strain (SAC). CD4+ T cell clones, expressing CD40L on their surface, also did not rescue IgG or IgA induction by X-HIM peripheral blood B cells in vitro. But signaling through CD40 induced both B cell proliferation and IgE secretion when IL-4 was added to the culture. Taken together, these results show that in vitro signaling through CD40 rescues IgE but not IgG or IgA secretion by peripheral blood X-HIM B cells and suggest that in vivo CD40 and CD40L interaction might be necessary for IgG and IgA differentiation in X-HIM.

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