Defective NKT cell development in mice and humans lacking the adapter SAP, the X-linked lymphoproliferative syndrome gene product.
Defective NKT cell development in mice and humans lacking the adapter SAP, the X-linked lymphoproliferative syndrome gene product.
复制标题
小鼠和人类缺乏适配器SAP的NKT细胞发育有缺陷，X连锁淋巴增生性综合征基因产物。
DOI：
10.1084/jem.20042432
复制
发表时间：
2005-03-07
期刊：
影响因子：
--
通讯作者：
Latour S
中科院分区：
文献类型：
--
作者：
Pasquier B;Yin L;Fondanèche MC;Relouzat F;Bloch-Queyrat C;Lambert N;Fischer A;de Saint-Basile G;Latour S
SAP是在T细胞和自然杀伤细胞中表达的衔接蛋白。它在免疫中起着关键作用，因为它在患有X连锁淋巴增生综合征（XLP）的人类中发生突变，这是一种致命的免疫缺陷，其特征是对EB病毒（EBV）感染的异常反应。SAP与SLAM家族受体相互作用，并通过其募集和激活Src激酶FynT的能力促进这些受体的转导信号事件。由于先前已经确定FynT是NKT细胞发育所需的选择性因子，因此我们在SAP缺陷小鼠和患有XLP的人类中检查了NKT细胞。在没有SAP的情况下，小鼠和人类的NKT细胞发育都严重受损。这些结果表明SAP是NKT细胞发育的有效调节剂。他们还首次发现了与人类原发性免疫缺陷相关的NKT细胞缺陷，揭示了NKT细胞在对EBV的免疫反应中的潜在作用。
SAP is an adaptor protein expressed in T cells and natural killer cells. It plays a critical role in immunity, as it is mutated in humans with X-linked lymphoproliferative syndrome (XLP), a fatal immunodeficiency characterized by an abnormal response to Epstein-Barr virus (EBV) infection. SAP interacts with the SLAM family receptors and promotes transduction signal events by these receptors through its capacity to recruit and activate the Src kinase FynT. Because it has been previously established that FynT is selectively required for the development of NKT cells, we examined NKT cells in SAP-deficient mice and in humans with XLP. In the absence of SAP, the development of NKT cells is severely impaired both in mice and in humans. These results imply that SAP is a potent regulator of NKT cell development. They also identify for the first time a defect in NKT cells associated with a human primary immunodeficiency, revealing a potential role of NKT cells in the immune response to EBV.
登录
查看更多内容
影响因子：
64.8
作者：
Crotty, S;Kersh, EN;Ahmed, R
通讯作者：
Ahmed, R
影响因子：
15.3
作者：
Benlagha, K;Weiss, A;Beavis, A;Teyton, L;Bendelac, A
通讯作者：
Bendelac, A
影响因子：
12.7
作者：
Yin, L;Al-Alem, U;Romeo, G
通讯作者：
Romeo, G
影响因子：
32.4
作者：
Davidson, D;Shi, XC;Veillette, A
通讯作者：
Veillette, A
影响因子：
16.8
作者：
Mars, LT;Novak, J;Lehuen, A
通讯作者：
Lehuen, A