Mouse NFKB2 blocking peptide

Most of primary immunodeficiencies with hypogammaglobulinemia are associated with reduced memory B cells. T cell development may be interesting as well, but increased recent thymic emigrants are rarely reported in these patients. We report the case of a family (mother and her two sons) diagnosed with common variable immunodeficiency 10 due to a mutation in the NFKB2 gene. Laboratory findings showed that all three patients presented hypogammaglobulinemia, reduced memory B cells and elevated naive T lymphocytes and recent thymic emigrants. This feature, in the absence of glucocorticoid deficiency, may suggest a primary thymic dysfunction. Interestingly, the mother presented the worst immune phenotype, as regards both antibody production and NK function, indicating that immune function may deteriorate in the course of time. We conclude that close monitoring of immune functions may widen the knowledge on the CVID10 and improve the patients' care.

The Epstein-Barr virus (EBV) latent membrane protein (LMP1) is believed to play a crucial role in oncogenesis mediated by this virus. We and others previously showed that LMP1 can induce NF kappa B activity in several non-lymphoid cells and B-lymphoid cell lines. Here we show that LMP1 is also able to efficiently induce NF kappa B in human T-lymphoid and monocytic cells. Specific NF kappa B complexes were detected in the nuclei of transfected Jurkat cells using gel mobility shift assays and Western blot analyses; Using antibodies, we demonstrated that these complexes contain NF kappa B subunits NFkB1, NFkB2, RelA and c-Rel. Our results also showed that the NF kappa B complexes induced by LMP1 are able to bind to the NF kappa B consensus sequence in the promoter of the interleukin-2 alpha receptor gene and induce expression from a minimal promoter linked to four tandem copies of this sequence. This suggests a possible mechanism by which LMP1 could induce T-cell activation and proliferation. (C) 2000 Elsevier Science B.V. All rights reserved.