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  4. RAG2 Antibody, FITC conjugated

RAG2 Antibody, FITC conjugated

  • 中文名稱：
    RAG2兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA345128LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) RAG2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
  • 別名：
    RAG 2 antibody; RAG-2 antibody; RAG2 antibody; RAG2_HUMAN antibody; Recombination activating gene 2 antibody; V(D)J recombination activating protein 2 antibody; V(D)J recombination-activating protein 2 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human V(D)J recombination-activating protein 2 protein (296-415AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    FITC
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    >95%, Protein G purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 產品提供形式：
    Liquid
  • 儲存條件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 貨期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
  • 用途：
    For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

  • 功能：
    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.
  • 基因功能參考文獻：
    1. Mutation of serine 365 to alanine permits bi-allelic and bi-locus RAG-mediated breaks in the same cell, leading to reciprocal translocations. PMID: 29069605
    2. the ability of the RAG nuclease to minimize the risks of genome disruption by coupling the breakage and repair steps of the V(D)J reaction. This implies that the RAG genes, derived from an ancient transposon, have undergone strong selective pressure to prohibit transposition in favor of promoting controlled DNA end joining in cis by the ubiquitous DNA damage response and DNA repair machineries PMID: 27863852
    3. The show that DNA damage caused by RAG2 activity in pre-B cells was able to downmodulate RAG2 expression and activity, confirming the existence of a negative feedback regulatory mechanism. PMID: 27559048
    4. gene deficiency results in late onset autoimmune hemolytic anemia PMID: 26515615
    5. DNA damage triggers relocalization of RAG2 from the nucleus to centrosomes, suggesting a novel mechanism for modulating cellular responses to double strand breaks in developing lymphocytes. PMID: 25625798
    6. found that Ikaros, a lymphocyte-specific transcription factor, acts as a repressor of NWC promoter--thus identifying a new Ikaros target--but is insufficient for inducing methylation which depends on the antisense transcription driven by RAG-2 promoter PMID: 25198102
    7. The results indicate that the contribution of immune dysregulatory disease due to RAG2 mutations present in the general population may be much higher than previously estimated. PMID: 24472623
    8. Bidirectional activity of the NWC promoter is responsible for RAG-2 transcription in non-lymphoid cells PMID: 22984564
    9. A novel homozygous mutation with different clinical phenotypes: Omenn syndrome and hyper-IgM syndrome PMID: 22841008
    10. analysis of multiorgan metastasis of human HER-2+ breast cancer in Rag2-/-;Il2rg-/- mice and treatment with PI3K inhibitor PMID: 22737248
    11. USF-1 but not USF-2 is strongly enriched at Dbeta2 in chromatin from either Rag2-/- deficient thymocytes or Rag2-/- deficient thymocytes that express a rearranged Tcrb transgene. PMID: 22287717
    12. Analysis of mutations from SCID and Omenn syndrome patients reveals the central role of the Rag2 PHD domain in regulating V(D)J recombination. PMID: 20234091
    13. No sequence changes in RAG2 were found in a series of hematologic leukemias or their cell lines. PMID: 12145704
    14. RAG2 expression and non-functional TCR rearrangements continuously take place in peripheral mature T cells of all activation/differentiation stages PMID: 12355431
    15. intrinsic RAG-2 NLS functions in the nuclear uptake of RAG-2 following its reexpression in cycling cells PMID: 12861017
    16. DNA methylation inhibits the cleavage activity of the RAG1/RAG2 complex by two different mechanisms, depending on the position of the mCpG around the recombination signal sequences PMID: 12897800
    17. Activated mature CD5-positive human tonsil B cells coexpress both RAG2 and RAG1 mRNA and protein, and display DNA cleavage resulting from their recombinase activity. PMID: 15843554
    18. SKP2 mediates ubiquitylation of RAG-2 in vitro and degradation of RAG-2 in vivo. PMID: 15949444
    19. The carboxyl terminus of Rag2 plays a unique role as a direct bridge between chromatin and recombinase during chromosomal V(D)J recombination PMID: 16111638
    20. A knockin murine model carrying the Rag2 R229Q mutation previously described in several patients with Omenn syndrome (OS)supports the notion that impaired immune tolerance and defective immune regulation are involved in the pathophysiology of OS. PMID: 17476358
    21. IL-6 initiates the expression of RAG2 in circulating B cells, and extends those in tonsil B cells. PMID: 17982069
    22. a conserved tryptophan residue (W453) that constitutes a key structural component of the K4me3-binding surface and is essential for RAG2's recognition of H3K4me3 is mutated in patients with immunodeficiency syndromes PMID: 18033247

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  • 相關疾病：
    Combined cellular and humoral immune defects with granulomas (CHIDG); Severe combined immunodeficiency autosomal recessive T-cell-negative/B-cell-negative/NK-cell-positive (T(-)B(-)NK(+) SCID); Omenn syndrome (OS)
  • 亞細胞定位：
    Nucleus.
  • 蛋白家族：
    RAG2 family
  • 組織特異性：
    Cells of the B- and T-lymphocyte lineages.
  • 數據庫鏈接：

    HGNC: 9832

    OMIM: 179616

    KEGG: hsa:5897

    STRING: 9606.ENSP00000308620

    UniGene: Hs.714519



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