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COQ4 Antibody

  • 货号：
    CSB-PA896885LA01HU
  • 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-PA896885LA01HU at dilution of 1:100
    • Immunohistochemistry of paraffin-embedded human spleen tissue using CSB-PA896885LA01HU at dilution of 1:100
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) COQ4 Polyclonal antibody
  • Uniprot No.：
    Q9Y3A0
  • 基因名：
    COQ4
  • 别名：
    CGI 92 antibody; Coenzyme Q biosynthesis protein 4 homolog antibody; coq4 antibody; COQ4_HUMAN antibody; mitochondrial antibody; Ubiquinone biosynthesis protein COQ4 homolog antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial protein (31-265AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，COQ4 Antibody (CSB-PA896885LA01HU)，的标记方式是Non-conjugated。对于COQ4 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-PA896885LB01HU COQ4 Antibody, HRP conjugated ELISA
    FITC CSB-PA896885LC01HU COQ4 Antibody, FITC conjugated
    Biotin CSB-PA896885LD01HU COQ4 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：
    Component of the coenzyme Q biosynthetic pathway. May play a role in organizing a multi-subunit COQ enzyme complex required for coenzyme Q biosynthesis. Required for steady-state levels of other COQ polypeptides.
  • 基因功能参考文献：
    1. The COQ4 mutation was CRISPR/Cas9 edited resulting in isogenic, diploid and off-target free COQ4-corrected iPSCs. PMID: 28465093
    2. Three genes in our epilepsy cohort (COQ4, DNM1, and PURA), accounting for 14% (3/21) of all novel genetic etiologies identified in patients with epilepsy, were subsequently confirmed in independent publications. PMID: 26795593
    3. five recessive missense mutations in COQ4 segregating with lethal neonatal mitochondrial encephalomyopathy in four families of Ashkenzi Jews PMID: 26185144
    4. COQ4 mutations cause a broad spectrum of mitochondrial disorders associated with CoQ10 deficiency. PMID: 25658047
    5. Haploinsufficiency in COQ4 resulted in reduced COQ4 expression, CoQ10 content and biosynthetic rate, and activities of respiratory chain complex II+III. PMID: 22368301
    6. Human ortholog of Saccharomyces cerevisiae COQ4 was cloned and characterized. PMID: 18474229
    7. homologous to Coq4p in S.cerevisiae PMID: 11469793

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  • 相关疾病：
    Coenzyme Q10 deficiency, primary, 7 (COQ10D7)
  • 亚细胞定位：
    [Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    COQ4 family
  • 组织特异性：
    Expressed ubiquitously, but at high levels in liver, lung and pancreas.
  • 数据库链接：

    HGNC: 19693

    OMIM: 612898

    KEGG: hsa:51117

    STRING: 9606.ENSP00000300452

    UniGene: Hs.98541