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ISCA1 Antibody

  • 货号：
    CSB-PA887126LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Immunohistochemistry of paraffin-embedded human liver tissue using CSB-PA887126LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) ISCA1 Polyclonal antibody
  • Uniprot No.：
    Q9BUE6
  • 基因名：
    ISCA1
  • 别名：
    GK004 antibody; HESB-like domain-containing protein 2 antibody; hIscA antibody; Iron sulfur assembly protein IscA antibody; Iron-sulfur assembly protein IscA antibody; Iron-sulfur cluster assembly 1 homolog antibody; Iron-sulfur cluster assembly 1 homolog, mitochondrial antibody; ISCA1 antibody; ISCA1_HUMAN antibody; mitochondrial antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Iron-sulfur cluster assembly 1 homolog, mitochondrial protein (67-122AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，ISCA1 Antibody (CSB-PA887126LA01HU)，的标记方式是Non-conjugated。对于ISCA1 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-PA887126LB01HU ISCA1 Antibody, HRP conjugated ELISA
    FITC CSB-PA887126LC01HU ISCA1 Antibody, FITC conjugated
    Biotin CSB-PA887126LD01HU ISCA1 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：
    Involved in the maturation of mitochondrial 4Fe-4S proteins functioning late in the iron-sulfur cluster assembly pathway. Probably involved in the binding of an intermediate of Fe/S cluster assembly.
  • 基因功能参考文献：
    1. the phenotype observed in all affected subjects with the ISCA1 pathogenic variant is similar to that previously described in all four types of multiple mitochondrial dysfunctions syndromes. PMID: 28356563
    2. The structural plasticity of the dimeric state of the [2Fe-2S] bound form of human GRX5 is the crucial factor that allows an efficient cluster transfer to the partner proteins human ISCA1 and ISCA2 by a specific protein-protein recognition mechanism. PMID: 24733926
    3. The data presented here show that the Isu1 suppressor mimics the frataxin effects on Nfs1, explaining the bypassing activity. PMID: 24217246
    4. ISCA1, ISCA2, and IBA57 were depleted by RNA interference. Depleted cells contained massively swollen and enlarged mitochondria that were virtually devoid of cristae membranes, demonstrating the importance of these proteins for mitochondrial biogenesis. PMID: 22323289
    5. Iron-binding activity of human iron-sulfur cluster assembly protein hIscA1 PMID: 20302570
    6. screening a human brain cDNA expression library with serum from a patient suffering from the autoimmune Sjogren's syndrome yielded a gene product called hIscA implicated in iron-sulfur cluster biogenesis PMID: 15262227
    7. Required for functional heme biosynthesis in erythroid cells. PMID: 19656490

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  • 相关疾病：
    Multiple mitochondrial dysfunctions syndrome 5 (MMDS5)
  • 亚细胞定位：
    Mitochondrion.
  • 蛋白家族：
    HesB/IscA family
  • 组织特异性：
    Detected in cerebellum, kidney and heart.
  • 数据库链接：

    HGNC: 28660

    OMIM: 611006

    KEGG: hsa:81689

    STRING: 9606.ENSP00000365159

    UniGene: Hs.449291