Your Good Partner in Biology Research

  1. 首頁
  2. 蛋白
  3. 重組蛋白
  4. Recombinant Human Chaperone activity of bc1 complex-like, mitochondrial (ADCK3), partial

Recombinant Human Chaperone activity of bc1 complex-like, mitochondrial (ADCK3), partial

  • 中文名稱：
    Recombinant Human Chaperone activity of bc1 complex-like, mitochondrial(ADCK3) ,partial,Yeast
  • 貨號：
    CSB-YP850918HU
  • 規(guī)格：
  • 來源：
    Yeast
  • 其他：
  • 中文名稱：
    Recombinant Human Chaperone activity of bc1 complex-like, mitochondrial(ADCK3) ,partial,Yeast
  • 貨號：
    CSB-EP850918HU
  • 規(guī)格：
  • 來源：
    E.coli
  • 其他：
  • 中文名稱：
    Recombinant Human Chaperone activity of bc1 complex-like, mitochondrial(ADCK3) ,partial,Yeast
  • 貨號：
    CSB-EP850918HU-B
  • 規(guī)格：
  • 來源：
    E.coli
  • 共軛：
    Avi-tag Biotinylated

    E. coli biotin ligase (BirA) is highly specific in covalently attaching biotin to the 15 amino acid AviTag peptide. This recombinant protein was biotinylated in vivo by AviTag-BirA technology, which method is BriA catalyzes amide linkage between the biotin and the specific lysine of the AviTag.

  • 其他：
  • 中文名稱：
    Recombinant Human Chaperone activity of bc1 complex-like, mitochondrial(ADCK3) ,partial,Yeast
  • 貨號：
    CSB-BP850918HU
  • 規(guī)格：
  • 來源：
    Baculovirus
  • 其他：
  • 中文名稱：
    Recombinant Human Chaperone activity of bc1 complex-like, mitochondrial(ADCK3) ,partial,Yeast
  • 貨號：
    CSB-MP850918HU
  • 規(guī)格：
  • 來源：
    Mammalian cell
  • 其他：

產(chǎn)品詳情

  • 純度：
    >85% (SDS-PAGE)
  • 基因名：
    COQ8A
  • Uniprot No.：
  • 別名：
    aarF domain containing protein kinase 3; aarF domain-containing protein kinase 3; ADCK 3; Adck3; ADCK3_HUMAN; ARCA2; CABC 1; CABC1 ; Chaperone ABC1 (activity of bc1 complex S.pombe) like; Chaperone ABC1 activity of bc1 complex homolog; Chaperone ABC1 like; Chaperone activity of bc1 complex like; Chaperone activity of bc1 complex like mitochondrial; Chaperone activity of bc1 complex-like; Chaperone-ABC1-like; Coenzyme Q8 homolog; COQ 8; COQ10D4; COQ8; COQ8; S. cerevisiae; homolog of; MGC4849; mitochondrial; OTTHUMP00000035734; OTTHUMP00000035737; SCAR9
  • 種屬：
    Homo sapiens (Human)
  • 蛋白長度：
    Partial
  • 表達區(qū)域：
    -
  • 蛋白標簽：
    Tag?type?will?be?determined?during?the?manufacturing?process.
    The tag type will be determined during production process. If you have specified tag type, please tell us and we will develop the specified tag preferentially.
  • 產(chǎn)品提供形式：
    Lyophilized powder
    Note: We will preferentially ship the format that we have in stock, however, if you have any special requirement for the format, please remark your requirement when placing the order, we will prepare according to your demand.
  • 復(fù)溶：
    We recommend that this vial be briefly centrifuged prior to opening to bring the contents to the bottom. Please reconstitute protein in deionized sterile water to a concentration of 0.1-1.0 mg/mL.We recommend to add 5-50% of glycerol (final concentration) and aliquot for long-term storage at -20℃/-80℃. Our default final concentration of glycerol is 50%. Customers could use it as reference.
  • 儲存條件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保質(zhì)期：
    The shelf life is related to many factors, storage state, buffer ingredients, storage temperature and the stability of the protein itself.
    Generally, the shelf life of liquid form is 6 months at -20°C/-80°C. The shelf life of lyophilized form is 12 months at -20°C/-80°C.
  • 貨期：
    Delivery time may differ from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
    Note: All of our proteins are default shipped with normal blue ice packs, if you request to ship with dry ice, please communicate with us in advance and extra fees will be charged.
  • 注意事項：
    Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
  • Datasheet ：
    Please contact us to get it.

產(chǎn)品評價

靶點詳情

  • 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：
    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

    顯示更多

    收起更多

  • 相關(guān)疾?。?/div>
    Coenzyme Q10 deficiency, primary, 4 (COQ10D4)
  • 亞細胞定位：
    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)庫鏈接：

    HGNC: 16812

    OMIM: 606980

    KEGG: hsa:56997

    STRING: 9606.ENSP00000355739

    UniGene: Hs.118241