Bibliographic Details
| Title: |
Homozygous loss of ICOS is associated with adult-onset common variable immunodeficiency. |
| Authors: |
Grimbacher, Bodo, Hutloff, Andreas, Schlesier, Michael, Glocker, Erik, Warnatz, Klaus, Drager, Ruth, Eibel, Hermann, Fischer, Beate, Schaffer, Alejandro A., Mages, Hans W., Kroczek, Richard A., Peter, Hans H. |
| Source: |
Nature Immunology. Mar2003, Vol. 4 Issue 3, p261. 8p. |
| Subject Terms: |
*IMMUNODEFICIENCY, *AGAMMAGLOBULINEMIA, *T cells |
| Abstract: |
No genetic defect is known to cause common variable deficiency (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[sup -/-] mouse, suggests a critical involvement of ICOS in T cell help for late B cell differentiation, class-switching and memory B cell generation. [ABSTRACT FROM AUTHOR] |
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| Database: |
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