CD267 Antibody

Tumor necrosis factor receptor superfamily member 13B (CD267) is a receptor for TNFSF13/APRIL and TNFSF13B/TALL1/BAFF/BLYS that binds both ligands with similar high affinity. CD267 mediates calcineurin-dependent activation of NF-AT, as well as activation of NF-kappa-B and AP-1. CD267 is involved in the stimulation of B- and T-cell function and the regulation of humoral immunity. Defects in CD267 are the cause of immunodeficiency common variable type 2 (CVID2). CVID2 is a primary immunodeficiency characterized by antibody deficiency, hypogammaglobulinemia, recurrent bacterial infections and an inability to mount an antibody response to antigen. The defect results from a failure of B-cell differentiation and impaired secretion of immunoglobulins. The numbers of circulating B cells is usually in the normal range, but can be low.