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MOCS2 Antibody, FITC conjugated

  • 中文名稱:
    MOCS2兔多克隆抗體, FITC偶聯
  • 貨號:
    CSB-PA014706LC01HU
  • 規格:
    ¥880
  • 其他:

產品詳情

  • 產品名稱:
    Rabbit anti-Homo sapiens (Human) MOCS2 Polyclonal antibody
  • Uniprot No.:
  • 基因名:
    MOCS2
  • 別名:
    MOCS2 antibody; MCBPE antibody; MOCO1 antibody; Molybdopterin synthase catalytic subunit antibody; EC 2.8.1.12 antibody; MOCO1-B antibody; Molybdenum cofactor synthesis protein 2 large subunit antibody; Molybdenum cofactor synthesis protein 2B antibody; MOCS2B antibody; Molybdopterin-synthase large subunit antibody; MPT synthase large subunit antibody
  • 宿主:
    Rabbit
  • 反應種屬:
    Human
  • 免疫原:
    Recombinant Human Molybdopterin synthase catalytic subunit protein (1-188AA)
  • 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:
    Catalytic subunit of the molybdopterin synthase complex, a complex that catalyzes the conversion of precursor Z into molybdopterin. Acts by mediating the incorporation of 2 sulfur atoms from thiocarboxylated MOCS2A into precursor Z to generate a dithiolene group.
  • 基因功能參考文獻:
    1. analysis of sites within MOCS2 where mutations cause molybdenum cofactor deficiency PMID: 12732628
    2. There was undisturbed production of both transcripts, while Western blot analysis demonstrated that MOCS2B, the large subunit, is unstable in the absence of MOCS2A. PMID: 16737835
    3. We reported here an infant with MoCD who presented with hypoxic ischaemic encephalopathy and identified a novel mutation, c.130C>T in cDNA of the MOCS2 gene from the infant. PMID: 17158010
  • 相關疾病:
    Molybdenum cofactor deficiency, complementation group B (MOCODB)
  • 亞細胞定位:
    Cytoplasm, cytosol.
  • 蛋白家族:
    MoaE family, MOCS2B subfamily
  • 組織特異性:
    Highest levels are found in heart and skeletal muscle. Lower levels are present in brain, kidney and pancreas. Very low levels are found in lung and peripheral blood leukocytes.
  • 數據庫鏈接:

    HGNC: 7193

    OMIM: 252160

    KEGG: hsa:4338

    STRING: 9606.ENSP00000380157

    UniGene: Hs.163645



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