The role of SAP and SLAM family molecules in the humoral immune response

Authors

  • Cindy S. Ma,

    1. Immunology Program, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia.
    2. St Vincent's Clinical School, University of NSW, Kensington, NSW, Australia
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  • Elissa K. Deenick

    1. Immunology Program, Garvan Institute of Medical Research, Darlinghurst, NSW, Australia.
    2. St Vincent's Clinical School, University of NSW, Kensington, NSW, Australia
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Address for correspondence: Elissa Deenick, Garvan Institute of Medical Research, 384 Victoria Street, Darlinghurst, NSW 2010, Australia. e.deenick@garvan.org.au

Abstract

Effective B cell–mediated immunity, including the formation of germinal centers and the generation of high-affinity memory B cells and long-lived plasma cells, is dependent on CD4+ T cells. Immunodeficiencies that present with defects in the antibody response have provided insights into the molecular mechanisms of B cell responses and the provision of T cell help. One such immunodeficiency is X-linked lymphoproliferative disease (XLP), which results from mutations in SH2D1A, the gene encoding SLAM-associated protein (SAP). Patients with XLP present with humoral defects characterized by hypogammaglobulinemia. We now know that SAP, through its signaling downstream of multiple members of the signaling lymphocytic activation molecule (SLAM) family of cell surface receptors, plays a crucial role in many aspects of this immune response. Here, we discuss the role of SAP in the generation of humoral immunity, particularly T cell–dependent antibody responses and the generation of germinal centers.

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