Schwartzberg Pamela L, Mueller Kristen L, Qi Hai, Cannons Jennifer L
National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland 20892, USA.
Nat Rev Immunol. 2009 Jan;9(1):39-46. doi: 10.1038/nri2456.
Mutations that affect the adaptor molecule SLAM-associated protein (SAP) underlie the primary immunodeficiency disease X-linked lymphoproliferative syndrome. SAP is required for mediating signals from members of the signalling lymphocytic activation molecule (SLAM) family of immunomodulatory receptors. Recent data have highlighted a role for SAP in the development of innate-like T-cell lineages, including natural killer T cells, and in the regulation of the interactions between B cells and T cells that are required for germinal-centre formation and long-term humoral immunity. These data have revealed that SLAM family members and SAP have crucial roles in regulating lymphocyte interactions and adhesion, which are required for the normal development, homeostasis and function of the immune system.
影响衔接分子信号淋巴细胞激活分子相关蛋白（SAP）的突变是原发性免疫缺陷病X连锁淋巴增殖综合征的基础。介导来自免疫调节受体信号淋巴细胞激活分子（SLAM）家族成员的信号需要SAP。最近的数据突出了SAP在先天性样T细胞谱系（包括自然杀伤T细胞）发育中的作用，以及在生发中心形成和长期体液免疫所需的B细胞与T细胞相互作用调节中的作用。这些数据表明，SLAM家族成员和SAP在调节淋巴细胞相互作用和黏附中起关键作用，而这是免疫系统正常发育、稳态和功能所必需的。
