SH2D1A (XLP 1D12) Rat mAb

• 产品编号：CST-2805S      品牌：CST       原厂货号：2805S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

SH2D1A (XLP 1D12) Rat mAb 能够检测内源SH2D1A蛋白总体水平。此单克隆抗体是通过合成融合的全长SH2D1A蛋白来免疫动物而获得。SH2D1A 和 SH2D1B 是小分子的接头蛋白， 有一个SH2域，在介导淋巴细胞的激活分子(SLAM)家族的信号受体中起了重要的信号转导作用(1)。SH2D1A (也叫做SAP或SLAM相关蛋白)在患有X-连锁的淋巴组织增生性疾病（邓肯氏病）的 病人中通常是突变的,这种疾病通常对Epstein-Barr病毒极度的敏感易感; 到目前为止报道了将近50个不同的SH2D1A 突变(2-4)。在人的基因组中有只有单个SH2D1B基因(也叫做EAT-2 或Ewing's sarcoma's/FLI1激活转录2)，在小鼠和大鼠基因组中此基因有一个复制即EAT-2A 和 EAT-2B基因，他们在基因组上有相同的组织方式(5,6)。

1. Latour, S. and Veillette, A. (2004) Semin. Immunol. 16, 409-419.
2. Nichols, K.E. et al. (2005) Immunol. Rev. 203, 180-199.
3. Engel, P. et al. (2003) Nat. Rev. Immunol. 3, 813-821.
4. Sumegi, J. et al. (2002) Leuk. Lymphoma 43, 1189-1201.
5. Roncagalli, R. et al. (2005) Nat. Immunol. 6, 1002-1010.
6. Calpe, S. et al. (2006) Immunogenetics 58, 15-25.

原厂资料：

Specificity / Sensitivity

SH2D1A (XLP 1D12) Rat mAb detects endogenous levels of total SH2D1A protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with a recombinant full-length SH2D1A protein.

Background

SH2D1A and SH2D1B are small, adaptor proteins with a single SH2-domain that play important signal transduction roles mediated by the signaling lymphocytic activation molecule (SLAM) family receptors (1). SH2D1A (also called SAP or SLAM-associated protein) is frequently mutated in patients with X-linked lymphoproliferative disease (Duncan’s disease), which is characterized by extreme susceptibility to Epstein-Barr virus; approximately 50 different SH2D1A mutations have been reported to date (2-4). The single SH2D1B gene in humans (also called EAT-2 or Ewing's sarcoma's/FLI1-activated transcript 2) is present as a pair of duplicated EAT-2A and EAT-2B genes with identical genomic organization in mouse and rat (5,6).

1. Latour, S. and Veillette, A. (2004) Semin. Immunol. 16, 409-419.
2. Nichols, K.E. et al. (2005) Immunol. Rev. 203, 180-199.
3. Engel, P. et al. (2003) Nat. Rev. Immunol. 3, 813-821.
4. Sumegi, J. et al. (2002) Leuk. Lymphoma 43, 1189-1201.
5. Roncagalli, R. et al. (2005) Nat. Immunol. 6, 1002-1010.
6. Calpe, S. et al. (2006) Immunogenetics 58, 15-25.

注意事项：

Storage: Supplied in 10 mM sodium HEPES (pH 7.5), 150
mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02%
sodium azide. Store at –20°C. Do not aliquot the antibody.