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  4. UNC80 Antibody, FITC conjugated

UNC80 Antibody, FITC conjugated

  • 中文名稱：
    UNC80兔多克隆抗體, FITC偶聯
  • 貨號：
    CSB-PA854025LC01HU
  • 規格：
    ￥880
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) UNC80 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    UNC80
  • 別名：
    C2orf21 antibody; Protein unc-80 homolog antibody; UNC 80 antibody; Unc 80 homolog (C. elegans) antibody; Unc80 antibody; UNC80_HUMAN antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human Protein unc-80 homolog protein (124-388AA)
  • 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：
    Component of the NALCN sodium channel complex, required for channel regulation. This complex is a cation channel activated by neuropeptides substance P, neurotensin, and extracellular calcium that regulates neuronal excitability by controlling the sizes of NALCN-dependent sodium-leak current. UNC80 is essential for NALCN sensitivity to extracellular calcium.
  • 基因功能參考文獻：
    1. UNC80 variant is associated with neurodevelopmental diseases. PMID: 30167850
    2. Biallelic UNC80 mutations caused infantile hypotonia with psychomotor retardation and characteristic facies 2 in two Chinese patients with variable phenotypes PMID: 29572195
    3. UNC80 bridges between UNC79 and the cation channel NALCN. PMID: 26545877
    4. Further supporting the UNC80 mutations as causative of these siblings' disorder, biallelic mutations in UNC80 have recently been described among individuals with an overlapping phenotype. This report expands the disease spectrum associated with UNC80 mutations PMID: 27513830
    5. findings demonstrate the fundamental significance of UNC80 and basal ionic conductance to human health PMID: 26708751
    6. UNC80 encodes a large protein that is necessary for the stability and function of NALCN and for bridging NALCN to UNC79 to form a functional complex PMID: 26708753
    7. UNC80 functions as a scaffold for Src kinases in NALCN channel function. PMID: 19535918

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  • 相關疾?。?/div>
    Hypotonia, infantile, with psychomotor retardation and characteristic facies 2 (IHPRF2)
  • 亞細胞定位：
    Membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Unc-80 family
  • 組織特異性：
    Moderately expressed in fetal brain, spinal cord, skeletal muscle, thymus, spleen, fetal liver, small intestine, colon, kidney and uterus. Highly expressed in adrenal gland, prostate and testis, as well as in brain and cerebellum.
  • 數據庫鏈接：

    HGNC: 26582

    OMIM: 612636

    KEGG: hsa:285175

    STRING: 9606.ENSP00000391088

    UniGene: Hs.396201



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