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CFHR5 Antibody

  • 货号：
    CSB-PA883624LA01HU
  • 规格：
    ￥440
  • 促销：
    小规格抗体限时一口价
  • 图片：
    • Western Blot
      Positive WB detected in: K562 whole cell lysate, HepG2 whole cell lysate, Hela whole cell lysate
      All lanes: CFHR5 antibody at 1:1000
      Secondary
      Goat polyclonal to rabbit IgG at 1/50000 dilution
      Predicted band size: 65 kDa
      Observed band size: 65 kDa
    • IHC image of CSB-PA883624LA01HU diluted at 1:100 and staining in paraffin-embedded human kidney tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
  • 其他：

产品详情

  • 产品名称：
    Rabbit anti-Homo sapiens (Human) CFHR5 Polyclonal antibody
  • Uniprot No.：
    Q9BXR6
  • 基因名：
    CFHR5
  • 别名：
    CFHL5 antibody; CFHR5 antibody; CFHR5D antibody; Complement factor H-related 5 antibody; Complement factor H-related protein 5 antibody; factor H-related gene 5 antibody; factor H-related protein 5 antibody; FHR-5 antibody; FHR5 antibody; FHR5_HUMAN antibody; FLJ10549 antibody; MGC133240 antibody; OTTHUMP00000034672 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Recombinant Human Complement factor H-related protein 5 protein (374-569AA)
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated

    本页面中的产品，CFHR5 Antibody (CSB-PA883624LA01HU)，的标记方式是Non-conjugated。对于CFHR5 Antibody，我们还提供其他标记。见下表：

    可提供标记
    标记方式 货号 产品名称 应用
    HRP CSB-PA883624LB01HU CFHR5 Antibody, HRP conjugated ELISA
    FITC CSB-PA883624LC01HU CFHR5 Antibody, FITC conjugated
    Biotin CSB-PA883624LD01HU CFHR5 Antibody, Biotin conjugated ELISA
  • 克隆类型：
    Polyclonal
  • 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：
    Preservative: 0.03% Proclin 300
    Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, WB, IHC
  • 推荐稀释比：
    Application Recommended Dilution
    WB 1:500-1:2000
    IHC 1:20-1:200
  • 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：
    Involved in complement regulation. The dimerized forms have avidity for tissue-bound complement fragments and efficiently compete with the physiological complement inhibitor CFH.
  • 基因功能参考文献：
    1. Novel genetic rearrangement generated from a heterozygous deletion spanning 146 Kbp involving multiple CFHR genes leading to a CFHR1-R5 hybrid protein. This deletion was found in four family members presenting with a familial dominant glomerulopathy. PMID: 28729035
    2. Higher serum FHR-5 levels were associated with a lack of response to immunosuppression, the presence of endocapillary hypercellularity, and histology scores of IgA nephropathy severity. PMID: 28673452
    3. Our study found that rare variants in CFHR5 may contribute to the genetic susceptibility to IgA Nephropathy, which suggests that CFHR5 is an IgA Nephropathy susceptibility gene PMID: 26825529
    4. Next-generation sequencing of the CFH region identified putatively functional variants (missense, splice site and indel) on the four common haplotypes. We found no expression of any of the five CFH-related genes in the retina or RPE/Choroid/Sclera, in contrast to the liver, which is the main source of the circulating proteins. [CFHR5] PMID: 27196323
    5. Studies indicate that complement factor H-related proteins (FHR1-5) may enhance complement activation, with important implications for the role of these proteins in disease. PMID: 25979655
    6. In this study, we identify pentraxin 3 (PTX3) as a novel ligand of CFHR5 PMID: 25855355
    7. At least two distinct intronic breakpoints within the CFHR5 gene can cause the same mutant CFHR5 protein and C3 glomerulopathy. PMID: 24067434
    8. A hybrid CFHR2-CFHR5 plasma protein, arising from a chromosomal deletion mutation stabilizes the C3 convertase and reduces factor H-mediated convertase decay. PMID: 24334459
    9. Recent investigations in London and Cyprus culminated in the identification of another autosomal dominant condition that presents with microscopic haematuria because of heterozygous mutations in the CFHR5 gene--{review} PMID: 23402027
    10. A potentially pathogenic sequence variation was found in CFHR5 in the patients with atypical hemolytic uremic syndrome. PMID: 22622361
    11. CFHR5 nephropathy is discussed. PMID: 22065842
    12. Describe the clinical course, significant variable expressivity, and marked gender difference regarding the development of chronic renal failure in familial C3 glomerulopathy associated with CFHR5 mutations. PMID: 21566112
    13. evidence for an inherited renal disease, endemic in Cyprus, characterised by microscopic and synpharyngitic macroscopic haematuria, renal failure and C3 glomerulonephritis; affected individuals have an internal duplication within the gene for CFHR5 PMID: 20800271
    14. Study identified novel mutations in CFH, CFHR5, CFI, CFB and C3 in American patients with atypical hemolytic uremic syndrome. PMID: 20513133
    15. Maps to between FHR-2 and the non-complement protein factor XIIIb at 1q32. PMID: 12041828
    16. FHR-5 shares properties of binding heparin and C-reactive protein and lipoprotein association with one or more of the other FHRs, but is unique among this family of proteins in possessing independent complement-regulatory activity. PMID: 15879123
    17. Identification of specific variants of variants of CFHR5 in membranoproliferative glomerulonephritis type II. PMID: 16299065
    18. CFHR5 genetic alterations may play a secondary role in the pathogenesis of haemolytic uraemic syndrome. PMID: 17000000
    19. No definitive pathogenic CFHR5 mutations have been found in any of 639 unrelated patients with age-related macular degeneration (AMD), indicating that sequence variations in CFHR5 do not play a major role in determining AMD susceptibility. PMID: 19365580

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  • 相关疾病：
    CFHR5 deficiency (CFHR5D)
  • 亚细胞定位：
    Secreted.
  • 组织特异性：
    Expressed by the liver and secreted in plasma.
  • 数据库链接：

    HGNC: 24668

    OMIM: 608593

    KEGG: hsa:81494

    STRING: 9606.ENSP00000256785

    UniGene: Hs.282594