FITC标记的重组激活基因2蛋白抗体
产品名称： FITC标记的重组激活基因2蛋白抗体
英文名称： Anti-RAG2/FITC
产品编号： HZ-6960R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： IF=1:50-200
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Rabbit Anti-RAG2/FITC Conjugated antibody
FITC标记的重组激活基因2蛋白抗体
| 产品编号 | bs-6960R-FITC |
| 英文名称 | Anti-RAG2/FITC |
| 中文名称 | FITC标记的重组激活基因2蛋白抗体 |
| 别 名 | RAG 2; RAG-2; RAG2; RAG2_HUMAN; Recombination activating gene 2; V(D)J recombination activating protein 2; V(D)J recombination-activating protein 2. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 免疫学 表观遗传学 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep, |
| 产品应用 | IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 58kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human RAG2 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: Catalytic component of the RAG complex, a multiprotein complex that mediates the DNA cleavage phase during V(D)J recombination. V(D)J recombination assembles a diverse repertoire of immunoglobulin and T-cell receptor genes in developing B and T lymphocytes through rearrangement of different V (variable), in some cases D (diversity), and J (joining) gene segments. In the RAG complex, RAG1 mediates the DNA-binding to the conserved recombination signal sequences (RSS) and catalyzes the DNA cleavage activities by introducing a double-strand break between the RSS and the adjacent coding segment. RAG2 is not a catalytic component but is required for all known catalytic activities. DNA cleavage occurs in 2 steps: a first nick is introduced in the top strand immediately upstream of the heptamer, generating a 3'-hydroxyl group that can attack the phosphodiester bond on the opposite strand in a direct transesterification reaction, thereby creating 4 DNA ends: 2 hairpin coding ends and 2 blunt, 5'-phosphorylated ends. The chromatin structure plays an essential role in the V(D)J recombination reactions and the presence of histone H3 trimethylated at 'Lys-4' (H3K4me3) stimulates both the nicking and haipinning steps. The RAG complex also plays a role in pre-B cell allelic exclusion, a process leading to expression of a single immunoglobulin heavy chain allele to enforce clonality and monospecific recognition by the B-cell antigen receptor (BCR) expressed on individual B lymphocytes. The introduction of DNA breaks by the RAG complex on one immunoglobulin allele induces ATM-dependent repositioning of the other allele to pericentromeric heterochromatin, preventing accessibility to the RAG complex and recombination of the second allele. In addition to its endonuclease activity, RAG1 also acts as a E3 ubiquitin-protein ligase that mediates monoubiquitination of histone H3. Histone H3 monoubiquitination is required for the joining step of V(D)J recombination. Mediates polyubiquitination of KPNA1. Function: Core component of the RAG complex, a multiprotein complex that mediates the DNA cleavage phase during V(D)J recombination. V(D)J recombination assembles a diverse repertoire of immunoglobulin and T-cell receptor genes in developing B and T-lymphocytes through rearrangement of different V (variable), in some cases D (diversity), and J (joining) gene segments. DNA cleavage by the RAG complex occurs in 2 steps: a first nick is introduced in the top strand immediately upstream of the heptamer, generating a 3'-hydroxyl group that can attack the phosphodiester bond on the opposite strand in a direct transesterification reaction, thereby creating 4 DNA ends: 2 hairpin coding ends and 2 blunt, 5'-phosphorylated ends. The chromatin structure plays an essential role in the V(D)J recombination reactions and the presence of histone H3 trimethylated at 'Lys-4' (H3K4me3) stimulates both the nicking and haipinning steps. The RAG complex also plays a role in pre-B cell allelic exclusion, a process leading to expression of a single immunoglobulin heavy chain allele to enforce clonality and monospecific recognition by the B-cell antigen receptor (BCR) expressed on individual B-lymphocytes. The introduction of DNA breaks by the RAG complex on one immunoglobulin allele induces ATM-dependent repositioning of the other allele to pericentromeric heterochromatin, preventing accessibility to the RAG complex and recombination of the second allele. In the RAG complex, RAG2 is not the catalytic component but is required for all known catalytic activities mediated by RAG1. It probably acts as a sensor of chromatin state that recruits the RAG complex to H3K4me3. Subunit: Component of the RAG complex composed of core components RAG1 and RAG2, and associated component HMGB1 or HMGB2. Subcellular Location: Nucleus. DISEASE: Defects in RAG2 are a cause of combined cellular and humoral immune defects with granulomas (CHIDG) [MIM:233650]. CHIDG is an immunodeficiency disease with granulomas in the skin, mucous membranes, and internal organs. Other characteristics include hypogammaglobulinemia, a diminished number of T and B-cells, and sparse thymic tissue on ultrasonography. Defects in RAG2 are a cause of severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-negative/NK-cell-positive (T(-)B(-)NK(+) SCID) [MIM:601457]. A form of severe combined immunodeficiency (SCID), a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-mediated immunity, leukopenia, and low or absent antibody levels. Patients present in infancy recurrent, persistent infections by opportunistic organisms. The common characteristic of all types of SCID is absence of T-cell-mediated cellular immunity due to a defect in T-cell development. Defects in RAG2 are a cause of Omenn syndrome (OS) [MIM:603554]. OS is a severe immunodeficiency characterized by the presence of activated, anergic, oligoclonal T-cells, hypereosinophilia, and high IgE levels. Similarity: Belongs to the RAG2 family. Contains 1 PHD-type zinc finger. Database links: Entrez Gene: 5897 Human Entrez Gene: 19374 Mouse Entrez Gene: 295953 Rat Omim: 179616 Human SwissProt: P55895 Human SwissProt: P21784 Mouse SwissProt: P34089 Rabbit Unigene: 714519 Human Unigene: 4988 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
RG复合物的催化组分，是在V（D）J重组期间介导DNA切割相的多蛋白复合物。V（D）J重组通过重组不同的V（可变），在某些情况下D（多样性）和J（连接）基因片段，在发育B和T淋巴细胞中组装免疫球蛋白和T细胞受体基因的多样性。在RAG复合体中，RAG1介导DNA与保守重组信号序列（RSS）的结合，并通过引入RSS和相邻编码片段之间的双链断裂来催化DNA切割活性。RAG2不是催化组分，但对所有已知的催化活性都是必需的。DNA裂解发生在2个步骤中：第一个缺口被引入到正庚烷上游的顶部链中，生成3’-羟基，该基团可在直接酯交换反应中攻击相反链上的磷酸二酯键，从而产生4个DNA末端：2个发夹编码EN。DS和2钝化，5’-磷酸化末端。染色质结构在V（D）J重组反应中起着至关重要的作用，在Lys4’（H3K4Me3）存在三甲基化组蛋白H3，刺激刻蚀和HIPIPIN步骤。RAG复合物在前B细胞等位基因排斥中起作用，这一过程导致单个B淋巴细胞抗原受体（BCR）在单个B淋巴细胞上表达克隆性和单特异性识别，以增强单个免疫球蛋白重链等位基因的表达。在一个免疫球蛋白等位基因上引入RAG复合体的DNA断裂诱导ATM依赖的另一个等位基因重新定位到周边着丝粒异染色质，从而阻止对第二等位基因的RAG复合体和重组的可访问性。除了其内切核酸酶活性外，RAG1还充当E3泛素蛋白连接酶，介导组蛋白H3的单自由基化。V（D）J重组的连接步骤需要Histone H3单环化。介导KpNa1的多聚泛素化。