概述

  • 产品名称Anti-RAG1抗体
    参阅全部 RAG1 一抗
  • 描述
    兔多克隆抗体to RAG1
  • 经测试应用适用于: WBmore details
  • 种属反应性
    与反应: Mouse
    预测可用于: Rat, Horse, Human, Pig
  • 免疫原

    Synthetic peptide corresponding to a region within C terminal amino acids 946- 995(

    IIERDGSIGAWASEGNESGNKLFRRFRKMNARQSKCYEMEDVLKHHWLYT

    ) of Mouse RAG1 (NP_033045).

  • 阳性对照
    • Mouse liver lysate

性能

应用

Our Abpromise guarantee covers the use of ab113821 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

应用 Ab评论 说明
WB Use a concentration of 1 µg/ml. Detects a band of approximately 119 kDa. Good results were obtained when blocked with 5% non-fat dry milk in 0.05% PBS-T.

靶标

  • 功能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.
  • 组织特异性Maturing lymphoid cells.
  • 疾病相关Defects in RAG1 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 RAG1 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 RAG1 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.
    Defects in RAG1 are the cause of alpha/beta T-cell lymphopenia with gamma/delta T-cell expansion severe cytomegalovirus infection and autoimmunity (T-CMVA) [MIM:609889]. An immunological disorder characterized by oligoclonal expansion of TCR gamma/delta T cells, TCR alpha/beta T cell lymphopenia, severe, disseminated cytomegalovirus infection and autoimmune cytopenia.
  • 序列相似性Belongs to the RAG1 family.
    Contains 1 NBD (nonamer binding) DNA-binding domain.
    Contains 1 RAG1-type zinc finger.
    Contains 1 RING-type zinc finger.
  • 结构域The RING-type zinc finger mediates the E3 ubiquitin-protein ligase activity.
    The NBD (nonamer binding) DNA-binding domain mediates the specific binding to the nonamer RSS motif by forming a tightly interwoven homodimer that binds and synapses 2 nonamer elements, with each NBD making contact with both DNA molecules. Each RSS is composed of well-conserved heptamer (consensus 5'-CACAGTG-3') and nonamer (consensus 5'-ACAAAAACC-3') sequences separated by a spacer of either 12 bp or 23 bp.
  • 翻译后修饰Autoubiquitinated in the presence of CDC34/UBCH3.
  • 细胞定位Nucleus.
  • Information by UniProt
  • 数据库链接
  • 别名
    • E3 ubiquitin-protein ligase RAG1 antibody
    • RAG-1 antibody
    • RAG1 antibody
    • RAG1_HUMAN antibody
    • recombination activating gene 1 antibody
    • recombination activating protein 1 antibody
    • RING finger protein 74 antibody
    • RNF74 antibody
    • V(D)J recombination-activating protein 1 antibody
    see all

Anti-RAG1 antibody 图像

  • Anti-RAG1 antibody (ab113821) at 1 µg/ml + Mouse liver lysate at 10 µg

Anti-RAG1 antibody (ab113821)参考文献

ab113821 has not yet been referenced specifically in any publications.

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