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Normal CD40-mediated activation of monocytes and dendritic cells from patients with hyper-IgM syndrome due to a CD40 pathway defect in B cells.

Authors :
Revy P
Geissmann F
Debré M
Fischer A
Durandy A
Source :
European journal of immunology [Eur J Immunol] 1998 Nov; Vol. 28 (11), pp. 3648-54.
Publication Year :
1998

Abstract

Patients with X-linked hyper-IgM syndrome [CD40 ligand (CD40L) deficiency] are prone to infections by intracellular parasites. It has been suggested that this susceptibility is caused by defective macrophage activation through the CD40L-CD40 pathway. We studied the CD40-mediated activation of monocytes and dendritic cells from patients affected with a CD40L+ hyper-IgM syndrome characterized by a defect of B lymphocyte responses to CD40 agonists. We show that the CD40-induced production of IL-6, IL-8 and TNF-alpha by monocytes, and IL-12 by dendritic cells, and expression of the activation markers CD83, the costimulatory molecules CD86 and CD80, and HLA-DR antigens were all similar in patient and control cells. This observation is consistent with the clinical characteristics of the syndrome: a defect of immunoglobulin switch but no susceptibility to opportunistic infections, as observed in CD40L-deficient patients. These observations suggest that CD40-mediated activation pathways could be, at least in part, different in B and monocytic/dendritic cell lineages.

Details

Language :
English
ISSN :
0014-2980
Volume :
28
Issue :
11
Database :
MEDLINE
Journal :
European journal of immunology
Publication Type :
Academic Journal
Accession number :
9842907
Full Text :
https://doi.org/10.1002/(SICI)1521-4141(199811)28:11<3648::AID-IMMU3648>3.0.CO;2-U