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  4. BOLA3 Antibody

BOLA3 Antibody

  • 中文名稱：
    BOLA3兔多克隆抗體
  • 貨號：
    CSB-PA690486LA01HU
  • 規格：
    ￥440
  • 圖片：
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA690486LA01HU at dilution of 1:100
  • 其他：

產品詳情

  • 產品名稱：
    Rabbit anti-Homo sapiens (Human) BOLA3 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    BOLA3
  • 別名：
    BolA-like protein 3 antibody; BOLA3 antibody; BOLA3_HUMAN antibody; MMDS2 antibody
  • 宿主：
    Rabbit
  • 反應種屬：
    Human
  • 免疫原：
    Recombinant Human BolA-like protein 3 protein (1-79AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標記方式：
    Non-conjugated

    本頁面中的產品，BOLA3 Antibody (CSB-PA690486LA01HU)，的標記方式是Non-conjugated。對于BOLA3 Antibody，我們還提供其他標記。見下表：

    可提供標記
    標記方式 貨號 產品名稱 應用
    HRP CSB-PA690486LB01HU BOLA3 Antibody, HRP conjugated ELISA
    FITC CSB-PA690486LC01HU BOLA3 Antibody, FITC conjugated
    Biotin CSB-PA690486LD01HU BOLA3 Antibody, Biotin conjugated ELISA
  • 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
  • 應用范圍：
    ELISA, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 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：
    Acts as a mitochondrial iron-sulfur (Fe-S) cluster assembly factor that facilitates (Fe-S) cluster insertion into a subset of mitochondrial proteins. Probably acts together with NFU1.
  • 基因功能參考文獻：
    1. Clinical features of BOLA3-associated variant nonketotic hyperglycemia include severe neurodegeneration after a period of normal development. PMID: 24334290
    2. BOLA3 plays a crucial role in the biogenesis of iron-sulfur clusters. PMID: 22562699
    3. Mutations in iron-sulfur cluster scaffold genes NFU1 and BOLA3 cause a fatal deficiency of multiple respiratory chain and 2-oxoacid dehydrogenase enzymes [case report] PMID: 21944046
    4. Clinical trial of gene-disease association and gene-environment interaction. (HuGE Navigator) PMID: 20379614
    5. This study reported that all three human BolA proteins (hBolA1, hBolA2, and hBolA3) are novel non-classical secreted proteins identified with bioinformatics and molecular biology experiments. PMID: 18548201
    6. BOLA-like proteins are widely conserved from prokaryotes to eukaryotes and may be involved in cell proliferation or cell-cycle regulation. PMID: 14718656

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  • 相關疾病：
    Multiple mitochondrial dysfunctions syndrome 2 with hyperglycinemia (MMDS2)
  • 亞細胞定位：
    Mitochondrion.
  • 蛋白家族：
    BolA/IbaG family
  • 組織特異性：
    Widely expressed.
  • 數據庫鏈接：

    HGNC: 24415

    OMIM: 613183

    KEGG: hsa:388962

    STRING: 9606.ENSP00000331369

    UniGene: Hs.61472



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