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  4. Recombinant Human Cytochrome c oxidase subunit 3 (MT-CO3)

Recombinant Human Cytochrome c oxidase subunit 3 (MT-CO3)

  • 货号：
    CSB-CF015074HU
  • 规格：
  • 来源：
    in vitro E.coli expression system
  • 其他：

产品详情

  • 基因名：
  • Uniprot No.：
  • 别名：
    MT-CO3; COIII; COXIII; MTCO3; Cytochrome c oxidase subunit 3; Cytochrome c oxidase polypeptide III
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    full length protein
  • 表达区域：
    1-261
  • 氨基酸序列
    MTHQSHAYHMVKPSPWPLTGALSALLMTSGLAMWFHFHSMTLLMLGLLTNTLTMYQWWRD VTRESTYQGHHTPPVQKGLRYGMILFITSEVFFFAGFFWAFYHSSLAPTPQLGGHWPPTG ITPLNPLEVPLLNTSVLLASGVSITWAHHSLMENNRNQMIQALLITILLGLYFTLLQASE YFESPFTISDGIYGSTFFVATGFHGLHVIIGSTFLTICFIRQLMFHFTSKHHFGFEAAAW YWHFVDVVWLFLYVSIYWWGS
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal 10xHis-tagged
  • 产品提供形式：
    Liquid or 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.
  • 缓冲液：
    Lyophilized from Tris/PBS-based buffer, 6% Trehalose, pH 8.0
  • 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：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. 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 & COA：
    Please contact us to get it.

产品评价

靶点详情

  • 功能：
    Component of the cytochrome c oxidase, the last enzyme in the mitochondrial electron transport chain which drives oxidative phosphorylation. The respiratory chain contains 3 multisubunit complexes succinate dehydrogenase (complex II, CII), ubiquinol-cytochrome c oxidoreductase (cytochrome b-c1 complex, complex III, CIII) and cytochrome c oxidase (complex IV, CIV), that cooperate to transfer electrons derived from NADH and succinate to molecular oxygen, creating an electrochemical gradient over the inner membrane that drives transmembrane transport and the ATP synthase. Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Electrons originating from reduced cytochrome c in the intermembrane space (IMS) are transferred via the dinuclear copper A center (CU(A)) of subunit 2 and heme A of subunit 1 to the active site in subunit 1, a binuclear center (BNC) formed by heme A3 and copper B (CU(B)). The BNC reduces molecular oxygen to 2 water molecules using 4 electrons from cytochrome c in the IMS and 4 protons from the mitochondrial matrix.
  • 基因功能参考文献：
    1. There was a significant association of cytochrome c oxidase III 15bp deletion with human male infertility. PMID: 26712170
    2. this is the first study showing to demonstrate that aa72-117 in HBx is the key region for binding with COXIII. PMID: 25483779
    3. A novel COIII gene m.9588G>A mutation was found in the mtDNA sperm's in all asthenozoospermic patients and was absent in the normozoospermic and in fertile men. PMID: 24550096
    4. novel heteroplasmic mutation m.9276G>C in the mitochondrial COIII gene, detected in mtDNA extracted from leukocytes of a mother and her two daughters indicating that this mutation is maternally transmitted PMID: 25701779
    5. Lack of Cox3 limits the biosynthesis of COX but does not alter the structure of the enzyme. PMID: 25588698
    6. A novel heteroplasmic missense mitochondrial mutation (m.9387 G>A) in COXIII gene found in 3 asthenospermic patients from Tunisia. PMID: 23645088
    7. A novel COX III subunit single base pair deletion is associated with a high number (45%) of COX-negative muscle fibers in a patient with severe rhabdomyolysis but no family history of neuromuscular disorder. PMID: 21163656
    8. a novel heteroplasmic missense mitochondrial mutation at nucleotide 9478 in COX3 gene in patients with Leigh syndrome PMID: 20525945
    9. The total mtDNA major arc deletion level was greater than the common deletion level in COX 3-deficient spiral ganglion cells. PMID: 20085441
    10. Data show that both normal and mutant alpha-synuclein specifically interact with the mitochondrial complex IV enzyme, cytochrome C oxidase (COX). PMID: 12059041
    11. Mutations in mtDNA-encoded cytochrome c oxidase subunit III genes causing isolated myopathy or severe encephalomyopathy. PMID: 16288875
    12. a MELAS syndrome Korean family harbor homoplasmic missense mutation in mitochondrial genom, which confers pathogenicity (Phe251Leu) PMID: 18587274

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  • 相关疾病：
    Leber hereditary optic neuropathy (LHON); Mitochondrial complex IV deficiency (MT-C4D); Recurrent myoglobinuria mitochondrial (RM-MT)
  • 亚细胞定位：
    Mitochondrion inner membrane; Multi-pass membrane protein.
  • 蛋白家族：
    Cytochrome c oxidase subunit 3 family
  • 数据库链接：

    HGNC: 7422

    OMIM: 220110

    KEGG: hsa:4514

    STRING: 9606.ENSP00000354982