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（Formulation）
Ascitic fluid containing 0.03% sodium azide,0.5% BSA, 50%glycerol.
稀释比例
Western Blot: 1/500 - 1/2000. ELISA: 1/10000. Not yet tested in other applications.
纯化工艺（Immunogen）
Affinity purification
背景（Background）
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-
蛋白名称
V(D)J recombination-activating protein 2
其他名称
RAG2; V(D)J recombination-activating protein 2; RAG-2
Fields
>>FoxO signaling pathway;>>Primary immunodeficiency
组织表达
Cells of the B- and T-lymphocyte lineages.
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