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  4. EARS2 Antibody, FITC conjugated

EARS2 Antibody, FITC conjugated

  • 中文名稱：
    EARS2兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA705509LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) EARS2 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    EARS2
  • 別名：
    3230401I01Rik antibody; AL024049 antibody; COXPD12 antibody; EARS 2 antibody; ears2 antibody; GluRS antibody; Glutamate--tRNA ligase antibody; Glutamyl tRNA synthetase 2 mitochondrial antibody; KIAA1970 antibody; mitochondrial antibody; mKIAA1970 antibody; MSE1 antibody; Probable glutamyl tRNA synthetase; mitochondrial antibody; Probable glutamyl-tRNA synthetase antibody; RGD1307904 antibody; SYEM_HUMAN antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Probable glutamate--tRNA ligase, mitochondrial protein (42-166AA)
  • 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：
    Catalyzes the attachment of glutamate to tRNA(Glu) in a two-step reaction: glutamate is first activated by ATP to form Glu-AMP and then transferred to the acceptor end of tRNA(Glu).
  • 基因功能參考文獻：
    1. This study presented that a new neurological disease, early-onset leukoencephalopathy with thalamus and brainstem involvement and high lactate, defined by a peculiar biphasic clinical course and caused by mutations in a single gene, EARS2. PMID: 22492562
  • 相關疾病：
    Combined oxidative phosphorylation deficiency 12 (COXPD12)
  • 亞細胞定位：
    Mitochondrion matrix.
  • 蛋白家族：
    Class-I aminoacyl-tRNA synthetase family, Glutamate--tRNA ligase type 1 subfamily
  • 數據庫鏈接：

    HGNC: 29419

    OMIM: 612799

    KEGG: hsa:124454

    STRING: 9606.ENSP00000343488

    UniGene: Hs.620541



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