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  4. COQ4 Antibody, FITC conjugated

COQ4 Antibody, FITC conjugated

  • 中文名稱：
    COQ4兔多克隆抗體, FITC偶聯(lián)
  • 貨號：
    CSB-PA896885LC01HU
  • 規(guī)格：
    ￥880
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) COQ4 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    COQ4
  • 別名：
    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
  • 反應(yīng)種屬：
    Human
  • 免疫原：
    Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial protein (31-265AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)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(chǎn)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(chǎn)品評價

靶點詳情

  • 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.
  • 基因功能參考文獻：
    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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  • 相關(guān)疾?。?/div>
    Coenzyme Q10 deficiency, primary, 7 (COQ10D7)
  • 亞細胞定位：
    [Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    COQ4 family
  • 組織特異性：
    Expressed ubiquitously, but at high levels in liver, lung and pancreas.
  • 數(shù)據(jù)庫鏈接：

    HGNC: 19693

    OMIM: 612898

    KEGG: hsa:51117

    STRING: 9606.ENSP00000300452

    UniGene: Hs.98541



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