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SCO1 Antibody

  • 货号：
    CSB-PA020852GA01HU
  • 规格：
    ￥3,900
  • 其他：

产品详情

  • Uniprot No.：
    O75880
  • 基因名：
    SCO1
  • 别名：
    Cytochrome oxidase deficient homolog antibody; Cytochrome oxidase deficient homolog 1 antibody; Protein SCO1 homolog mitochondrial antibody; Protein SCO1 homolog, mitochondrial antibody; SCO (cytochrome oxidase deficient yeast) homolog 1 antibody; SCO cytochrome oxidase deficient homolog 1 (yeast) antibody; SCO cytochrome oxidase deficient homolog 1 antibody; sco1 antibody; SCO1_HUMAN antibody; SCOD1 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Human SCO1
  • 免疫原种属：
    Homo sapiens (Human)
  • 抗体亚型：
    IgG
  • 纯化方式：
    Antigen Affinity Purified
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA,WB
  • 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：
    Copper metallochaperone essential for the maturation of cytochrome c oxidase subunit II (MT-CO2/COX2). Not required for the synthesis of MT-CO2/COX2 but plays a crucial role in stabilizing MT-CO2/COX2 during its subsequent maturation. Involved in transporting copper to the Cu(A) site on MT-CO2/COX2. Plays an important role in the regulation of copper homeostasis by controlling the abundance and cell membrane localization of copper transporter CTR1.
  • 基因功能参考文献：
    1. Results find that COA6 associates with COX2 and is crucial for its maturation and complex IV biogenesis. Also, COA6 interacts with the copper chaperone SCO1 which indicates that COA6 is intrinsically involved in the copper delivery process for COX2. PMID: 26160915
    2. Sco1 is a metallochaperone that selectively transfers Cu(I) ions based on loop recognition, whereas Sco2 is a copper-dependent thiol reductase of the cysteine ligands in the oxidase. PMID: 26351686
    3. COX20 cooperates with SCO1 and SCO2 to mature COX2 and promote the assembly of cytochrome c oxidase. PMID: 24403053
    4. COX19 is necessary for the transduction of a SCO1-dependent mitochondrial redox signal that regulates ATP7A-mediated cellular copper efflux. PMID: 23345593
    5. Results describe the tissue distribution of SCO1 and SCO2 in mouse and human tissues. PMID: 20864674
    6. SCO1 facilitates the transfer of copper from SCO2 to the CuA site at an early stage of COX assembly in mitochondria. PMID: 15229189
    7. data suggest that both Cu(I) and Cu(II) binding are critical for normal Sco function. PMID: 16091356
    8. Cox17-mediated copper metallation of Sco1, as well as the subsequent failure of Cu(A) site maturation, is the basis for the inefficient assembly of the cytochrome c oxidase complex in SCO1 patients PMID: 16520371
    9. Sco1 has evolved to bind a metal atom via the di-Cys motif to act as a copper chaperone, the oxidized form of the nickel-bound protein suggests that it may also maintain the thioredoxin function. PMID: 16735468
    10. These results suggest a mitochondrial pathway for the regulation of cellular copper content that involves signaling through SCO1 and SCO2, perhaps by their thiol redox or metal-binding state. PMID: 17189203
    11. Cu(I)HCox17(2S-S), i.e., the copper-loaded form of the protein, can transfer simultaneously copper(I) and two electrons to the human cochaperone Sco1 (HSco1) in the oxidized state, i.e., with its metal-binding cysteines forming a disulfide bond. PMID: 18458339
    12. a fraction of Sco1 physically associates with the cytochrome c oxidase complex in human muscle mitochondria, suggesting a possible direct relationship between CcO and the regulation of cellular copper homeostasis PMID: 19295170
    13. SCO2 acts upstream of SCO1, and that it is indispensable for CO II synthesis. PMID: 19336478

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  • 相关疾病：
    Mitochondrial complex IV deficiency (MT-C4D)
  • 亚细胞定位：
    Mitochondrion. Mitochondrion inner membrane; Single-pass membrane protein.
  • 蛋白家族：
    SCO1/2 family
  • 组织特异性：
    Predominantly expressed in tissues characterized by high rates of oxidative phosphorylation (OxPhos), including muscle, heart, and brain.
  • 数据库链接：

    HGNC: 10603

    OMIM: 220110

    KEGG: hsa:6341

    STRING: 9606.ENSP00000255390

    UniGene: Hs.14511