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  4. Recombinant Human Complement factor H-related protein 5 (CFHR5)

Recombinant Human Complement factor H-related protein 5 (CFHR5)

  • 货号：
    CSB-YP883624HU
  • 规格：
  • 来源：
    Yeast
  • 其他：
  • 货号：
    CSB-EP883624HU
  • 规格：
  • 来源：
    E.coli
  • 其他：
  • 货号：
    CSB-EP883624HU-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：
  • 货号：
    CSB-BP883624HU
  • 规格：
  • 来源：
    Baculovirus
  • 其他：
  • 货号：
    CSB-MP883624HU
  • 规格：
  • 来源：
    Mammalian cell
  • 其他：

产品详情

  • 纯度：
    >85% (SDS-PAGE)
  • 基因名：
    CFHR5
  • Uniprot No.：
  • 别名：
    CFHL5; CFHR5; CFHR5D; Complement factor H-related 5; Complement factor H-related protein 5; factor H-related gene 5; factor H-related protein 5; FHR-5; FHR5; FHR5_HUMAN; FLJ10549; MGC133240; OTTHUMP00000034672
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full Length of Mature Protein
  • 表达区域：
    19-569
  • 氨基酸序列
    EG TLCDFPKIHH GFLYDEEDYN PFSQVPTGEV FYYSCEYNFV SPSKSFWTRI TCTEEGWSPT PKCLRMCSFP FVKNGHSESS GLIHLEGDTV QIICNTGYSL QNNEKNISCV ERGWSTPPIC SFTKGECHVP ILEANVDAQP KKESYKVGDV LKFSCRKNLI RVGSDSVQCY QFGWSPNFPT CKGQVRSCGP PPQLSNGEVK EIRKEEYGHN EVVEYDCNPN FIINGPKKIQ CVDGEWTTLP TCVEQVKTCG YIPELEYGYV QPSVPPYQHG VSVEVNCRNE YAMIGNNMIT CINGIWTELP MCVATHQLKR CKIAGVNIKT LLKLSGKEFN HNSRIRYRCS DIFRYRHSVC INGKWNPEVD CTEKREQFCP PPPQIPNAQN MTTTVNYQDG EKVAVLCKEN YLLPEAKEIV CKDGRWQSLP RCVESTAYCG PPPSINNGDT TSFPLSVYPP GSTVTYRCQS FYKLQGSVTV TCRNKQWSEP PRCLDPCVVS EENMNKNNIQ LKWRNDGKLY AKTGDAVEFQ CKFPHKAMIS SPPFRAICQE GKFEYPICE
  • 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

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