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COQ6 Antibody

  • 中文名稱：
    COQ6兔多克隆抗體
  • 貨號(hào)：
    CSB-PA897098LA01HU
  • 規(guī)格：
    ￥440
  • 圖片：
    • Western Blot
      Positive WB detected in: Mouse heart tissue, Mouse liver tissue
      All lanes: COQ6 antibody at 3μg/ml
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      predicted band size: 51, 49, 43 kDa
      observed band size: 51, 49 kDa
    • Immunohistochemistry of paraffin-embedded human testis tissue using CSB-PA897098LA01HU at dilution of 1:100
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱：
    Rabbit anti-Homo sapiens (Human) COQ6 Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    COQ6
  • 別名：
    CGI-10 antibody; Coenzyme Q6 homolog (yeast) antibody; Coenzyme Q6 homolog; monooxygenase (S. cerevisiae) antibody; Coenzyme Q6 homolog; monooxygenase (yeast) antibody; coq6 antibody; COQ6_HUMAN antibody; Ubiquinone biosynthesis monooxygenase COQ6 antibody
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human, Mouse
  • 免疫原：
    Recombinant Human Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial protein (208-339AA)
  • 免疫原種屬：
    Homo sapiens (Human)
  • 標(biāo)記方式：
    Non-conjugated

    本頁(yè)面中的產(chǎn)品，COQ6 Antibody (CSB-PA897098LA01HU)，的標(biāo)記方式是Non-conjugated。對(duì)于COQ6 Antibody，我們還提供其他標(biāo)記。見下表：

    可提供標(biāo)記
    標(biāo)記方式 貨號(hào) 產(chǎn)品名稱 應(yīng)用
    HRP CSB-PA897098LB01HU COQ6 Antibody, HRP conjugated ELISA
    FITC CSB-PA897098LC01HU COQ6 Antibody, FITC conjugated
    Biotin CSB-PA897098LD01HU COQ6 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(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA, WB, IHC
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:5000
    IHC 1:20-1:200
  • Protocols：
  • 儲(chǔ)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(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-hexaprenyl-4-hydroxybenzoic acid (HHB) to 3-hexaprenyl-4,5-dihydroxybenzoic acid (DHHB). The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6. Is able to perform the deamination reaction at C4 of 3-hexaprenyl-4-amino-5-hydroxybenzoic acid (HHAB) to produce DHHB when expressed in yeast cells lacking COQ9, even if utilization of para-aminobenzoic acid (pABA) involving C4-deamination seems not to occur in bacteria, plants and mammals, where only C5 hydroxylation of HHB has been shown.
  • 基因功能參考文獻(xiàn)：
    1. Primary CoQ10 deficiency due to COQ6 mutations should be considered in children presenting with both Steroid-Resistant Focal Segmental Glomerulosclerosis and sensorineural hearing loss. An early diagnosis of COQ6 mutations is essential because the condition is treatable when CoQ10 supplementation is started at the early stage. PMID: 28117207
    2. Data indicate a heterozygous loss-of-function coenzyme Q10 (CoQ10) biosynthesis monooxygenase 6 gene (COQ6)missense mutation in familial schwannomatosis. PMID: 24763291
    3. COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness PMID: 21540551
  • 相關(guān)疾病：
    Coenzyme Q10 deficiency, primary, 6 (COQ10D6)
  • 亞細(xì)胞定位：
    Mitochondrion inner membrane; Peripheral membrane protein; Matrix side. Golgi apparatus. Cell projection.
  • 蛋白家族：
    UbiH/COQ6 family
  • 組織特異性：
    Widely expressed.
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 20233

    OMIM: 614647

    KEGG: hsa:51004

    STRING: 9606.ENSP00000333946

    UniGene: Hs.131555



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