RAG1 (D36B3) Rabbit mAb

• 产品编号：CST-3968S      品牌：CST       原厂货号：3968S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

RAG1 (D36B3) Rabbit mAb能够检测内源RAG1蛋白总体水平。此单克隆抗体是通过合成人源对应的RAG1蛋白的融合蛋白免疫动物而获得。在免疫细胞的发育过程中，编码配体受体的序列最终分化成多个种系片段，随后经过一个叫做V(D)J的重组过程。一个可变的片段(V) 与一个连接片段(J)相连接, 有时还有一个多样性片段(D), 他们共同组成了一个受体的配体结合部分。重组的V(D)J片段然后拼接到外显子编码的恒定区产生成熟mRNA(1,2)。这一重要进程对成熟的免疫T细胞核B细胞受体的多样性的发育是必需的(3,4)。启动这一进程包括RAG1 (重组活性基因1) 和 RAG2结合到保守的重组信号序列(RSS)，在RSS和编码区域引入一个双链的断裂(5,6)。RAG1和RAG2基因正好定位于基因组的连接位置，在很多物种中他们的编码区域缺乏内含子。RAG1和RAG2在T和B细胞中共表达并且是剪切活性所必需的(7)。RAG1和RAG2也具有转座子的功能, 有助于染色体易位和淋巴组织的恶性肿瘤(8,9)。RAG基因的突变体与很多人类的联合免疫缺陷疾病有关(10,11)。

1. Schatz, D.G. (2004) Semin Immunol 16, 245-56.
2. Gellert, M. (2002) Annu Rev Biochem 71, 101-32.
3. Alt, F.W. et al. (1992) Ann N Y Acad Sci 651, 277-94.
4. Sadofsky, M.J. (2001) Nucleic Acids Res 29, 1399-409.
5. Swanson, P.C. (2004) Immunol Rev 200, 90-114.
6. Swanson, P.C. et al. (2009) Adv Exp Med Biol 650, 1-15.
7. Fugmann, S.D. et al. (2000) Annu Rev Immunol 18, 495-527.
8. Hiom, K. et al. (1998) Cell 94, 463-70.
9. Agrawal, A. et al. (1998) Nature 394, 744-51.
10. Villa, A. et al. (1999) J Clin Immunol 19, 87-97.
11. Corneo, B. et al. (2000) J Biol Chem 275, 12672-5.

原厂资料：

Specificity / Sensitivity

RAG1 (D36B3) Rabbit mAb detects endogenous levels of total RAG1 protein.

Source / Purification

Monoclonal antibody is produced by immunizing animals with recombinant human RAG1 protein.

Background

The sequences encoding antigen receptors are split into multiple germline segments which are then combined by a process called V(D)J recombination during immune cells development. A variable (V) segment is combined with a joining (J) segment, and in some cases a D (Diversity) segment, to create the antigen-binding portion of the receptor. The recombined V(D)J segment is then spliced into exons that encode the constant region to produce mature mRNA (1,2). This essential process required for the development of functional immune T and B cells creates a vast diversity in these receptors (3,4). Initiation of this process follows binding of RAG1 (recombination activating gene 1) and RAG2 to the conserved recombination signal sequences (RSS) and the introduction of a double-strand break between the RSS and the coding sequence (5,6). RAG1 and RAG2 genes are located immediately adjacent to each other in the genome and lack introns in their coding regions in many species. RAG1 and RAG2 are coexpressed only in the B and T cell lineages and both are required for cleavage activity (7). RAG1 and RAG2 can also function as transposases, contributing to chromosomal translocations and lymphoid malignancy (8,9). Mutations in the RAG genes are associated with a spectrum of combined immune deficiencies in humans (10,11).

1. Schatz, D.G. (2004) Semin Immunol 16, 245-56.
2. Gellert, M. (2002) Annu Rev Biochem 71, 101-32.
3. Alt, F.W. et al. (1992) Ann N Y Acad Sci 651, 277-94.
4. Sadofsky, M.J. (2001) Nucleic Acids Res 29, 1399-409.
5. Swanson, P.C. (2004) Immunol Rev 200, 90-114.
6. Swanson, P.C. et al. (2009) Adv Exp Med Biol 650, 1-15.
7. Fugmann, S.D. et al. (2000) Annu Rev Immunol 18, 495-527.
8. Hiom, K. et al. (1998) Cell 94, 463-70.
9. Agrawal, A. et al. (1998) Nature 394, 744-51.
10. Villa, A. et al. (1999) J Clin Immunol 19, 87-97.
11. Corneo, B. et al. (2000) J Biol Chem 275, 12672-5.

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.