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QRSL1 Antibody

  • 中文名稱：
    QRSL1兔多克隆抗體
  • 貨號(hào)：
    CSB-PA887948ESR1HU
  • 規(guī)格：
    ￥440
  • 圖片：
    • Immunohistochemistry of paraffin-embedded human small intestine tissue using CSB-PA887948ESR1HU at dilution of 1:100
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) QRSL1 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    QRSL1
  • 別名：
    QRSL1 antibody; Glutamyl-tRNA(Gln) amidotransferase subunit A antibody; mitochondrial antibody; Glu-AdT subunit A antibody; EC 6.3.5.7 antibody; Glutaminyl-tRNA synthase-like protein 1 antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Glutamyl-tRNA(Gln) amidotransferase subunit A, mitochondrial protein (289-528AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated
  • 克隆類型：
    Polyclonal
  • 抗體亞型：
    IgG
  • 純化方式：
    Antigen Affinity Purified
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 保存緩沖液：
    PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 儲(chǔ)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(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    Allows the formation of correctly charged Gln-tRNA(Gln) through the transamidation of misacylated Glu-tRNA(Gln) in the mitochondria. The reaction takes place in the presence of glutamine and ATP through an activated gamma-phospho-Glu-tRNA(Gln).
  • 基因功能參考文獻(xiàn)：
    1. Homozygous intronic variant (c.850-3A > G) in the QRSL1 was identified in a child with cardiomyopathy with early-onset brain disease. PMID: 29440775
    2. Studies showed in vitro Gln-tRNA(Gln) formation catalyzed by the recombinant mtGluRS and hGatCAB. PMID: 19805282
  • 相關(guān)疾?。?/div>
    Defects in QRSL1 may play a role in mitochondrial disorders characterized by combined respiratory chain complex deficiencies.
  • 亞細(xì)胞定位：
    Mitochondrion.
  • 蛋白家族：
    Amidase family, GatA subfamily
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 21020

    KEGG: hsa:55278

    STRING: 9606.ENSP00000358042

    UniGene: Hs.406917