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ERCC6L2 Antibody

  • 货号：
    CSB-PA004261LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human lung tissue using CSB-PA004261LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) ERCC6L2 Polyclonal antibody
  • Uniprot No.：
    Q5T890
  • 基因名：
    ERCC6L2
  • 别名：
    C9orf102 antibody; Chromosome 9 open reading frame 102 antibody; excision repair cross-complementing rodent repair deficiency; complementation group 6-like 2 antibody; FLJ37706 antibody; MGC30192 antibody; MGC43364 antibody; Putative DNA repair and recombination protein RAD26-like antibody; putative repair and recombination helicase RAD26L antibody; RAD26_HUMAN antibody; Rad26l antibody; RAD26L hypothetical protein antibody; SR278 antibody; stretch responsive protein 278 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human DNA excision repair protein ERCC-6-like 2 protein (72-371AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，ERCC6L2 Antibody (CSB-PA004261LA01HU)，的标记方式是Non-conjugated。对于ERCC6L2 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA004261LB01HU ERCC6L2 Antibody, HRP conjugated ELISA
    FITC CSB-PA004261LC01HU ERCC6L2 Antibody, FITC conjugated
    Biotin CSB-PA004261LD01HU ERCC6L2 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 功能：
    May be involved in early DNA damage response.
  • 基因功能参考文献：
    1. ERCC6L2-associated disorder has recently been described. We identified an additional case through whole-exome sequencing. At the age of 9 years, the patient underwent whole exome sequencing and was discovered to have a homozygous stop mutation in ERCC6L2 (NCBI RefSeq NG_034107.1), c.1687C>T (p.Arg563*). PMID: 29633571
    2. The inherited bone marrow failure syndrome caused by biallelic variants in ERCC6L2 can be considered as a primary transcription deficiency rather than a DNA repair defect PMID: 29987015
    3. Hebo is ubiquitously expressed, localized in the nucleus, and rapidly recruited to DNAdsb's in an NBS1-dependent manner. PMID: 27185855
    4. These observations identify a distinct bone-marrow-failure syndrome due to mutations in ERCC6L2, a gene implicated in DNA repair and mitochondrial function. PMID: 24507776
  • 相关疾病：
    Bone marrow failure syndrome 2 (BMFS2)
  • 亚细胞定位：
    Nucleus. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome. Mitochondrion. Note=Colocalizes with NEK6 in the centrosome. In response to DNA damage, translocates from the cytosol to mitochondria and nucleus in a reactive oxygen species (ROS)-dependent manner.
  • 蛋白家族：
    SNF2/RAD54 helicase family
  • 组织特异性：
    Expressed in bone marrow (at protein level).
  • 数据库链接：

    HGNC: 26922

    OMIM: 615667

    KEGG: hsa:375748

    STRING: 9606.ENSP00000288985

    UniGene: Hs.432364