Your Good Partner in Biology Research

  1. Home
  2. 抗体
  3. 多克隆抗体
  4. ADCK3 Antibody

ADCK3 Antibody

  • 货号：
    CSB-PA973086
  • 规格：
    ￥2024
  • 图片：
    • Western blot analysis of extracts from HepG2 cells and mouse muscle cells, using ADCK3 antibody.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) COQ8A Polyclonal antibody
  • Uniprot No.：
    Q8NI60
  • 基因名：
    COQ8A
  • 宿主：
    Rabbit
  • 反应种属：
    Human,Mouse,Rat
  • 免疫原：
    Synthesized peptide derived from internal of Human ADCK3.
  • 免疫原种属：
    Homo sapiens (Human)
  • 克隆类型：
    Polyclonal
  • 纯化方式：
    The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:3000
  • 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：
    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.
  • 基因功能参考文献：
    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

    显示更多

    收起更多

  • 相关疾病：
    Coenzyme Q10 deficiency, primary, 4 (COQ10D4)
  • 亚细胞定位：
    Mitochondrion. Membrane; Single-pass membrane protein.
  • 蛋白家族：
    Protein kinase superfamily, ADCK protein kinase family
  • 组织特异性：
    Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle.
  • 数据库链接：

    HGNC: 16812

    OMIM: 606980

    KEGG: hsa:56997

    STRING: 9606.ENSP00000355739

    UniGene: Hs.118241