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SERPINB7 Antibody

  • 货号：
    CSB-PA070133
  • 规格：
    ￥880
  • 其他：

产品详情

  • Uniprot No.：
    O75635
  • 基因名：
    SERPINB7
  • 别名：
    Megsin antibody; Mesangium predominant gene megsin antibody; MGC120014 antibody; MGC120015 antibody; Serpin B7 antibody; Serpin peptidase inhibitor clade B (ovalbumin) member 7 antibody; Serpinb7 antibody; SPB7_HUMAN antibody; TP55 antibody
  • 宿主：
    Rabbit
  • 反应种属：
    Human
  • 免疫原：
    Synthesized peptide derived from the Internal region of Human Megsin.
  • 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 1:20000
  • 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：
    Might function as an inhibitor of Lys-specific proteases. Might influence the maturation of megakaryocytes via its action as a serpin.
  • 基因功能参考文献：
    1. SERPINB7 mutations are related strictly to the PPKN phenotype. Our study indicates that screening for SERPINB7 mutations is useful to distinguish PPKN from other PPK types and from non-PPK keratinizing diseases with palmoplantar skin lesions. PMID: 28211129
    2. Direct sequencing of SERPINB7 revealed five homozygous founder mutations (c.796C>T) and four compound heterozygous mutations in nine patients, including one novel mutation (c.122_127delTGGTCC). PMID: 27666198
    3. Case Report: striate palmoplantar keratoderma showing transgrediens in a patient with heterozygote nonsense mutations in DSG1 and SERPINB7. PMID: 27786350
    4. Results show splice variants from the two recurrent splice-site mutations in SERPINB7, provide evidence for the pathogenicity of the mutations and suggest an in-frame deletion of exon 3 may cause NPPK and that the CD-loop could affect SERPINB7 function PMID: 25940237
    5. These results indicate that megsin gene variants may play a role in the severity, development, and/or progression of IgAN in Northwest Chinese population PMID: 26871801
    6. Recessive missense mutation of SERPINB7 gene was found in Japanese families diagnosed with palmoplantar keratosis. PMID: 26763456
    7. Megsin 2093C/T TT genotype, and C25663G G allele and GG genotype were associated with the risk of IgAN in Asian population PMID: 25007157
    8. These data clearly provide further evidence that NPPK is caused by loss-of-function mutations in SERPINB7. PMID: 24773080
    9. megsin 2093C/T C allele may be genetic marker for IgA nephropathy susceptibility [review] PMID: 24575807
    10. The mean concentration of serpin B7 at 28-32 weeks was 1.5-fold higher in women with subsequent preterm deliveries compared to controls. PMID: 24954659
    11. study reports on seven unrelated Chinese patients with Nagashima-Type Palmoplantar Keratosis with four underlying SERPINB7 mutations, of which one is a recurrent variant (c.796C>T) PMID: 24514002
    12. All of the identified mutants are predicted to cause premature termination upstream of the reactive site, which inhibits the proteases, suggesting a complete loss of the protease inhibitory activity of SERPINB7 in palmoplantar keratosis skin. PMID: 24207119
    13. A267G in 5'-untranslated region within the exon of megsin may be one of the substantial genetic basis for differentiating "deficiency of liver yin and kidney yin" syndrome and "deficiency of qi and yin" syndrome in primary immunoglobulin A nephropathy. PMID: 18471408
    14. Mesangial cell-predominant gene, megsin. Genetic manipulation of megsin engenders two elementary mesangial lesions, mesangial expansion and an increase in the number of mesangial cells. PMID: 12386281
    15. One positive regulatory motif, an incomplete activator protein-1 binding motif (CTGATTCAC) within the -120 to -112 region. This cis-acting element in the 5'-flanking region of megsin is involved in the activation of the megsin gene in mesangial cells. PMID: 12397041
    16. Megsin has a role in susceptibility to immunoglobulin A nephropathy PMID: 15213261
    17. in transgenic rats, overexpression of human megsin, a recently discovered serpin located in the kidney, produces renal and pancreatic lesions characteristic of serpinopathies PMID: 15788472
    18. In this Chinese population, the 2093C-2180T haplotype at the 3'UTR of MEGSIN gene is associated with more severe forms of IgA nephropathy, and more rapid disease progression PMID: 16431886
    19. The polymorphism of megsin A23167G is associated with susceptibility and progression of IgA nephropathy in Chinese population. GG genotype is associated with severe histological lesions and progression of the disease. PMID: 16550745
    20. study found out that the megsin TT haplotype (defined as T-2093, T-2180 alleles) could play a protective role in the progression of IgA nephropathy PMID: 18498720
    21. recombinant megsin did not affect the mRNA expressions of TGF- and PAI-1, and did not modify the enzymatic activity of PAI-1 PMID: 18580857
    22. Megsin 2093T-2180C haplotype at the 3' untranslated region is associated with poor renal survival in Korean IgA nephropathy patients. PMID: 18793525

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  • 相关疾病：
    Keratoderma, palmoplantar, Nagashima type (PPKN)
  • 亚细胞定位：
    Cytoplasm.
  • 蛋白家族：
    Serpin family, Ov-serpin subfamily
  • 组织特异性：
    Predominantly expressed in mesangial cells. Expressed in the epidermis of the whole body.
  • 数据库链接：

    HGNC: 13902

    OMIM: 603357

    KEGG: hsa:8710

    STRING: 9606.ENSP00000337212

    UniGene: Hs.138202