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- Title
Homozygous loss of ICOS is associated with adult-onset common variable immunodeficiency.
- Authors
Grimbacher, Bodo; Hutloff, Andreas; Schlesier, Michael; Glocker, Erik; Warnatz, Klaus; Dräger, Ruth; Eibel, Hermann; Fischer, Beate; Schäffer, Alejandro A; Mages, Hans W; Kroczek, Richard A; Peter, Hans H
- Abstract
No genetic defect is known to cause common variable immunodeficiency (CVID), a heterogeneous human disorder leading to adult-onset panhypogammaglobulinemia. In a search for CVID candidate proteins, we found four of 32 patients to lack ICOS, the "inducible costimulator" on activated T cells, due to an inherited homozygous deletion in the ICOS gene. T cells from these individuals were normal with regard to subset distribution, activation, cytokine production and proliferation. In contrast, naive, switched and memory B cells were reduced. The phenotype of human ICOS deficiency, which differs in key aspects from that of the ICOS-/- mouse, suggests a critical involvement of ICOS in T cell help for late B cell differentiation, class-switching and memory B cell generation.
- Publication
Nature immunology, 2003, Vol 4, Issue 3, p261
- ISSN
1529-2908
- Publication type
Journal Article
- DOI
10.1038/ni902