Homozygous loss of ICOS is associated with adult-onset common variable immunodeficiency View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2003-03

AUTHORS

Bodo Grimbacher, Andreas Hutloff, Michael Schlesier, Erik Glocker, Klaus Warnatz, Ruth Dräger, Hermann Eibel, Beate Fischer, Alejandro A Schäffer, Hans W Mages, Richard A Kroczek, Hans H Peter

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. More... »

PAGES

261-268

Identifiers

URI

http://scigraph.springernature.com/pub.10.1038/ni902

DOI

http://dx.doi.org/10.1038/ni902

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1038439169

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/12577056


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