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  4. Cleaved-C1R (R463) Antibody

Cleaved-C1R (R463) Antibody

  • 货号：
    CSB-PA050282
  • 规格：
    ￥880
  • 其他：

产品详情

  • Uniprot No.：
    P00736
  • 基因名：
  • 别名：
    C1 R antibody; C1R antibody; C1R_HUMAN antibody; Complement C1r antibody; Complement C1r subcomponent antibody; Complement C1r subcomponent light chain antibody; Complement C1r subcomponent precursor antibody; Complement component 1 r subcomponent antibody; Complement component 1 subcomponent r antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthesized peptide derived from the Internal region of Human C1r HC.
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 抗体亚型：
    IgG
  • 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.
  • 保存缓冲液：
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • 产品提供形式：
    Liquid
  • 应用范围：
    WB, ELISA
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    ELISA 1:10000
  • 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：
    C1r B chain is a serine protease that combines with C1q and C1s to form C1, the first component of the classical pathway of the complement system.
  • 基因功能参考文献：
    1. The serine protease domains of C1r and C1s are at the periphery of the C1r2s2 tetramer both when alone or within the nonactivated C1 complex. The C1 complex adopts a conformation incompatible with intramolecular activation of C1. Instead, intermolecular proteolytic activation between neighboring C1 complexes bound to a complement-activating surface occurs. Many structurally unrelated molecular patterns can activate C1. PMID: 28104818
    2. We identified a novel, homozygous, loss-of-function mutation (p.Pro445Leufs*11) in the C1R gene. Using the Sanger method of DNA sequencing in 14 family members, we confirmed the presence of the mutation in 4 patients with early-onset systemic lupus erythematosus and in an asymptomatic 9-year-old girl. Complement levels were low in sera from patients with truncated C1r protein. PMID: 28544690
    3. Periodontal Ehlers-Danlos Syndrome in at least the great majority of cases results from specific classes of heterozygous mutations in C1R and C1S. PMID: 27745832
    4. We confirmed increased levels of C1R and VTN in sera from patients with Joint hypermobility syndrome by western blot analyses PMID: 26709396
    5. C1q exists as the C1 complex (C1qC1r2C1s2), and C1q binding to ligands activates the C1r/C1s proteases. Incubation of nucleoli with C1 caused degradation of the nucleolar proteins nucleolin and nucleophosmin 1. T PMID: 26231209
    6. C1r specificity is well suited to its cleavage targets and that efficient cleavage of C1s is achieved through both active site and exosite contributions. PMID: 23589288
    7. a structural rearrangement as a switch between functional states of human C1r PMID: 20970424
    8. These results provide further structural insights into the architecture of the C1 complex, and the interactions between C1r and C1s. PMID: 20592021
    9. The modular C1r protein is the first protease activated in the classical complement pathway, a key component of innate immunity. PMID: 20796027
    10. Detailed mapping of C1q post-translational modifications and insights into the C1r/C1s binding sites. PMID: 20008834
    11. Using a recombinant CUB2-CCP1 domain pair and the individual CCP1 module, we showed that binding of Ca(2+) induces the folding of the CUB2 domain and stabilizes its structure. PMID: 20178990
    12. These findings show no evidence for association between C1r codon 135 polymorphism and Alzheimer's Disease in our population. PMID: 12499050
    13. Six common and rare alleles, C1R*1, C1R*2, C1R*5, C1R*8, C1R*9, and C1R*13, were characterized by five mutations at amino acid positions 114, 135, 146, 167 and 244, in exons 4, 5 and 7 where the PMID: 12914573
    14. The activated CCP1-CCP2-SP fragment makes up a dimer in a head-to-tail fashion similarly to the previously characterized zymogen. PMID: 17996945
    15. The catalytic properties of C1r, the protease that mediates activation of the C1 complex of complement, are mediated by its C-terminal region, comprising two complement control protein (CCP) modules followed by a serine protease (SP) domain PMID: 11445589

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  • 相关疾病：
    Ehlers-Danlos syndrome, periodontal type, 1 (EDSPD1)
  • 亚细胞定位：
    Secreted.
  • 蛋白家族：
    Peptidase S1 family
  • 数据库链接：

    HGNC: 1246

    OMIM: 130080

    KEGG: hsa:715

    UniGene: Hs.524224