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  4. Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial (COQ4)

Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial (COQ4)

  • 中文名称：
    Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
  • 货号：
    CSB-YP896885HU
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 中文名称：
    Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
  • 货号：
    CSB-EP896885HU
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 中文名称：
    Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
  • 货号：
    CSB-EP896885HU-B
  • 规格：
  • 来源：
    E.coli
  • 共轭：
    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：
  • 中文名称：
    Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
  • 货号：
    CSB-BP896885HU
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 中文名称：
    Recombinant Human Ubiquinone biosynthesis protein COQ4 homolog, mitochondrial(COQ4)
  • 货号：
    CSB-MP896885HU
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
    COQ4
  • Uniprot No.：
    Q9Y3A0
  • 别名：
    CGI 92; Coenzyme Q biosynthesis protein 4 homolog; coq4; COQ4_HUMAN; mitochondrial; Ubiquinone biosynthesis protein COQ4 homolog
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    full length protein
  • 表达区域：
    31-265
  • 氨基酸序列
    SDGAGPLYSH HLPTSPLQKG LLAAGSAAMA LYNPYRHDMV AVLGETTGHR TLKVLRDQMR RDPEGAQILQ ERPRISTSTL DLGKLQSLPE GSLGREYLRF LDVNRVSPDT RAPTRFVDDE ELAYVIQRYR EVHDMLHTLL GMPTNILGEI VVKWFEAVQT GLPMCILGAF FGPIRLGAQS LQVLVSELIP WAVQNGRRAP CVLNLYYERR WEQSLRALRE ELGITAPPMH VQGLA
  • 蛋白标签：
    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.
  • 产品提供形式：
    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.
  • 复溶：
    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.
  • 储存条件：
    Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
  • 保质期：
    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.
  • 货期：
    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.
  • 注意事项：
    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.

产品评价

相关产品

COQ4 antibodies

靶点详情

  • 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.
  • 基因功能参考文献：
    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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  • 相关疾病：
    Coenzyme Q10 deficiency, primary, 7 (COQ10D7)
  • 亚细胞定位：
    [Isoform 1]: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
  • 蛋白家族：
    COQ4 family
  • 组织特异性：
    Expressed ubiquitously, but at high levels in liver, lung and pancreas.
  • 数据库链接：

    HGNC: 19693

    OMIM: 612898

    KEGG: hsa:51117

    STRING: 9606.ENSP00000300452

    UniGene: Hs.98541

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