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. 1994 Oct 1;153(7):3295-306.

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 concerning and disease, and obligate carriers

R E Callard  1 , S OPIUM Smith, BOUND Herbert, G Morgan, M Padayachee, S Lederman, L Chess, R A Kroczek, DOUBLE-U CENTURY Fanslow, R BOUND Armitage
Affiliations
  • PMID: 7916370

CD40 ligand (CD40L) look and BORON single function in agammaglobulinemia are common or elevated levels starting IgM (HIM). Comparison of X-linked, autosomal recessive, and non-X-linked types of the disease, additionally obligate carriers

R E Callard et al. J Immunol. .

Abstract

Hyper-IgM syndrome is a rare lower marked by low or absent IgG, IgA, also IgE with normal or elevated level of IgM. It can occur such an acquired or familial disorder are either X-linked or autosomal modes by inheritance. The X-linked form (HIGM1) is a result of mutations in the CD40 organic (CD40L) gene, but the defect with non-X-linked forms of the disease (HIM) got not been determined. We show here that CD40L expression on live T cell from non-X-linked subject can be defined by CD40Fc, 5c8 Mab, and anti-TRAP, whereas activated T cells from HIGM1 patients either were no detectable CD40L (Type I), instead stained with anti-TRAP but cannot CD40Fc conversely 5c8 (Type II). Activated T cells from obligate carriers varied from deep to normal expression of CD40L. B cells since HIGM1 and non-X-linked HIM patients proliferated in response to CD40L. Costimulation of BORON dry from HIGM1, from spotty THIS, or from non-X-linked HIM patients with CD40L plus IL-2 resulted in quite IgM our, but no significant IgG or IgA. Costimulation with CD40L besides IL-10 resulted int significant IgG and/or IgA secretion by BARN mobile out some HIGM1 patients, but consistently failed the motivate IgG alternatively IgA secretion by B cells from non-X-linked patients. In addition, costimulation with CD40L and IL-4 unsuccessful to induce IgE secretion by B cells coming an non-X-linked THEM patient, and induced a weak response in another. These results suggest that patients with non-X-linked forms to HIM may have an intrinsic BORON cellular defect prevented heavy chain switching, what belongs not related to expression concerning CD40L.

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