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CD40 ligand (CD40L) expression and B cell function in agammaglobulinemia with normal or elevated levels of IgM (HIM). Comparison of X-linked, autosomal recessive, and non-X-linked forms of the disease, and obligate carriers.

R E Callard, S H Smith, J Herbert, G Morgan, M Padayachee, S Lederman, L Chess, R A Kroczek, W C Fanslow and R J Armitage
J Immunol October 1, 1994, 153 (7) 3295-3306;
R E Callard
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S H Smith
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J Herbert
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G Morgan
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M Padayachee
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S Lederman
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L Chess
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R A Kroczek
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W C Fanslow
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R J Armitage
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Abstract

Hyper-IgM syndrome is a rare immunodeficiency characterized by low or absent IgG, IgA, and IgE with normal or elevated levels of IgM. It can occur as an acquired or familial disorder with either X-linked or autosomal modes of inheritance. The X-linked form (HIGM1) is a result of mutations in the CD40 ligand (CD40L) gene, but the defect in non-X-linked forms of the disease (HIM) has not been determined. We show here that CD40L expression on activated T cells from non-X-linked patients can be detected by CD40Fc, 5c8 Mab, and anti-TRAP, whereas activated T cells from HIGM1 patients either had no detectable CD40L (Type I), or stained with anti-TRAP but not CD40Fc or 5c8 (Type II). Activated T cells from obligate carriers varied from low to normal expression of CD40L. B cells from HIGM1 and non-X-linked HIM patients proliferated in response to CD40L. Costimulation of B cells from HIGM1, from sporadic HIM, or from non-X-linked HIM patients with CD40L plus IL-2 resulted in some IgM production, but no significant IgG or IgA. Costimulation with CD40L plus IL-10 resulted in significant IgG and/or IgA secretion by B cells from some HIGM1 patients, but consistently failed to stimulate IgG or IgA secretion by B cells from non-X-linked patients. In addition, costimulation with CD40L and IL-4 failed to induce IgE secretion by B cells from one non-X-linked HIM patient, and induced a weak response in another. These results suggest that patients with non-X-linked forms of HIM may have an intrinsic B cell defect preventing heavy chain switching, which is not related to expression of CD40L.

  • Copyright © 1994 by American Association of Immunologists

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The Journal of Immunology
Vol. 153, Issue 7
1 Oct 1994
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CD40 ligand (CD40L) expression and B cell function in agammaglobulinemia with normal or elevated levels of IgM (HIM). Comparison of X-linked, autosomal recessive, and non-X-linked forms of the disease, and obligate carriers.
R E Callard, S H Smith, J Herbert, G Morgan, M Padayachee, S Lederman, L Chess, R A Kroczek, W C Fanslow, R J Armitage
The Journal of Immunology October 1, 1994, 153 (7) 3295-3306;

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CD40 ligand (CD40L) expression and B cell function in agammaglobulinemia with normal or elevated levels of IgM (HIM). Comparison of X-linked, autosomal recessive, and non-X-linked forms of the disease, and obligate carriers.
R E Callard, S H Smith, J Herbert, G Morgan, M Padayachee, S Lederman, L Chess, R A Kroczek, W C Fanslow, R J Armitage
The Journal of Immunology October 1, 1994, 153 (7) 3295-3306;
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Print ISSN 0022-1767        Online ISSN 1550-6606