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Nat Immunol. 2003 Mar;4(3):261-8. Epub 2003 Feb 10.

Homozygous loss of ICOS is associated with adult-onset common variable immunodeficiency.

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Division of Rheumatology and Clinical Immunology, Medical School, University of Freiburg, Hugstetterstr. 55, 79106 Freiburg, Germany.


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.

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