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BOLA3 Antibody

  • 货号：
    CSB-PA690486LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human kidney tissue using CSB-PA690486LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) BOLA3 Polyclonal antibody
  • Uniprot No.：
    Q53S33
  • 基因名：
    BOLA3
  • 别名：
    BolA-like protein 3 antibody; BOLA3 antibody; BOLA3_HUMAN antibody; MMDS2 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human BolA-like protein 3 protein (1-79AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，BOLA3 Antibody (CSB-PA690486LA01HU)，的标记方式是Non-conjugated。对于BOLA3 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA690486LB01HU BOLA3 Antibody, HRP conjugated ELISA
    FITC CSB-PA690486LC01HU BOLA3 Antibody, FITC conjugated
    Biotin CSB-PA690486LD01HU BOLA3 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 抗体亚型：
    IgG
  • 纯化方式：
    >95%, Protein G purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    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.

产品评价

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Multiple mitochondrial dysfunctions syndrome 2 with hyperglycinemia (MMDS2)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    BolA/IbaG family
  • 组织特异性：
    Widely expressed.
  • 数据库链接：

    HGNC: 24415

    OMIM: 613183

    KEGG: hsa:388962

    STRING: 9606.ENSP00000331369

    UniGene: Hs.61472