RAG-2 Monoclonal Antibody
- 货号：YM0550
- 应用：WB;ELISA
- 种属：Human
- 简介：
- >>FoxO signaling pathway;>>Primary immunodeficiency
- 蛋白名称：
- V(D)J recombination-activating protein 2
- 免疫原：
- Purified recombinant fragment of human RAG-2 (350-527aa) expressed in E. Coli.
- 特异性：
- RAG-2 Monoclonal Antibody detects endogenous levels of RAG-2 protein.
- 组成：
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 稀释：
- WB 1:500 - 1:2000. ELISA: 1:10000. Not yet tested in other applications.
- 纯化工艺：
- Affinity purification
- 储存：
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 其他名称：
- RAG2;V(D)J recombination-activating protein 2;RAG-2
- 背景：
- This gene encodes a protein that is involved in the initiation of V(D)J recombination during B and T cell development. This protein forms a complex with the product of the adjacent recombination activating gene 1, and this complex can form double-strand breaks by cleaving DNA at conserved recombination signal sequences. The recombination activating gene 1 component is thought to contain most of the catalytic activity, while the N-terminal of the recombination activating gene 2 component is thought to form a six-bladed propeller in the active core that serves as a binding scaffold for the tight association of the complex with DNA. A C-terminal plant homeodomain finger-like motif in this protein is necessary for interactions with chromatin components, specifically with histone H3 that is trimethylated at lysine 4. Mutations in this gene cause Omenn syndrome, a form of severe combined immunodef
- 功能：
- 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.,disease:Defects in RAG2 are a cause of Omenn syndrome (OS) [MIM:603554]; a severe immunodeficiency characterized by the presence of activated, anergic, oligoclonal T-cells, hypereosinophilia, and high IgE levels.,disease: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]. SCID refers to a genetically and clinically heterogeneous group of rare congenital disorders characterized by impairment of both humoral and cell-
- 组织表达：
- Cells of the B- and T-lymphocyte lineages.
- Western Blot analysis using RAG-2 Monoclonal Antibody against RAG2-hIgGFc transfected HEK293 (1)cell lysate.
