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ADCK3 Antibody

  • 中文名稱(chēng)：
    ADCK3兔多克隆抗體
  • 貨號(hào)：
    CSB-PA973086
  • 規(guī)格：
    ￥2024
  • 圖片：
    • Western blot analysis of extracts from HepG2 cells and mouse muscle cells, using ADCK3 antibody.
  • 其他：

產(chǎn)品詳情

  • 產(chǎn)品名稱(chēng)：
    Rabbit anti-Homo sapiens (Human) COQ8A Polyclonal antibody
  • Uniprot No.：
  • 基因名：
    COQ8A
  • 宿主：
    Rabbit
  • 反應(yīng)種屬：
    Human,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from internal of Human ADCK3.
  • 免疫原種屬：
    Homo sapiens (Human)
  • 克隆類(lèi)型：
    Polyclonal
  • 純化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 濃度：
    It differs from different batches. Please contact us to confirm it.
  • 產(chǎn)品提供形式：
    Liquid
  • 應(yīng)用范圍：
    ELISA,WB
  • 推薦稀釋比：
    Application Recommended Dilution
    WB 1:500-1:3000
  • 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.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

  • 功能：
    Atypical kinase involved in the biosynthesis of coenzyme Q, also named ubiquinone, an essential lipid-soluble electron transporter for aerobic cellular respiration. Its substrate specificity is unclear: does not show any protein kinase activity. Probably acts as a small molecule kinase, possibly a lipid kinase that phosphorylates a prenyl lipid in the ubiquinone biosynthesis pathway, as suggested by its ability to bind coenzyme Q lipid intermediates. Shows an unusual selectivity for binding ADP over ATP.
  • 基因功能參考文獻(xiàn)：
    1. Loss of COQ8a results in cerebellar ataxia and coenzyme Q deficiency. PMID: 27499294
    2. ADCK3/COQ8 localises to mitochondrial cristae and is targeted to this organelle via the presence of an N-terminal localisation signal PMID: 26866375
    3. work reveals Mg(2+)-dependent ATPase activity of ADCK3, providing strong support for the theoretical prediction of this protein being a functional atypical kinase. PMID: 25540914
    4. a structural foundation for investigating the role of transmembrane association in regulating the biological activity of ADCK3 PMID: 25216398
    5. Mitochondrial ADCK3 employs an atypical protein kinase-like fold to enable coenzyme Q biosynthesis. PMID: 25498144
    6. These observations highlight the importance of screening for a potentially treatable cause, CABC1/ADCK3 mutations PMID: 22036850
    7. play an important role in mediating p53-inducible apoptosis through the mitochondrial pathway. PMID: 11888884
    8. CABC1 gene mutations in four ubiquinone-deficient patients in three distinct families were reported. PMID: 18319072
    9. Five additional mutations in ADCK3 were found in three patients with sporadic ataxia, including one known to have CoQ10 deficiency in muscle. PMID: 18319074

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  • 相關(guān)疾?。?/div>
    Coenzyme Q10 deficiency, primary, 4 (COQ10D4)
  • 亞細(xì)胞定位：
    Mitochondrion. Membrane; Single-pass membrane protein.
  • 蛋白家族：
    Protein kinase superfamily, ADCK protein kinase family
  • 組織特異性：
    Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle.
  • 數(shù)據(jù)庫(kù)鏈接：

    HGNC: 16812

    OMIM: 606980

    KEGG: hsa:56997

    STRING: 9606.ENSP00000355739

    UniGene: Hs.118241