Recombinant Human Serpin B7 (SERPINB7)

^{In Stock}

中文名稱：

人SERPINB7重組蛋白
貨號(hào)：

CSB-EP021075HU
規(guī)格：

￥1836
圖片：
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
其他：

產(chǎn)品詳情
產(chǎn)品評(píng)價(jià)
相關(guān)產(chǎn)品
靶點(diǎn)詳情

產(chǎn)品詳情

純度：

Greater than 85% as determined by SDS-PAGE.
基因名：

SERPINB7
Uniprot No.：

O75635
種屬：

Homo sapiens (Human)
蛋白長(zhǎng)度：

Full Length
來源：

E.coli
分子量：

69.6 kDa
表達(dá)區(qū)域：

1-380aa
氨基酸序列

MASLAAANAEFCFNLFREMDDNQGNGNVFFSSLSLFAALALVRLGAQDDSLSQIDKLLHVNTASGYGNSSNSQSGLQSQLKRVFSDINASHKDYDLSIVNGLFAEKVYGFHKDYIECAEKLYDAKVERVDFTNHLEDTRRNINKWVENETHGKIKNVIGEGGISSSAVMVLVNAVYFKGKWQSAFTKSETINCHFKSPKCSGKAVAMMHQERKFNLSVIEDPSMKILELRYNGGINMYVLLPENDLSEIENKLTFQNLMEWTNPRRMTSKYVEVFFPQFKIEKNYEMKQYLRALGLKDIFDESKADLSGIASGGRLYISRMMHKSYIEVTEEGTEATAATGSNIVEKQLPQSTLFRADHPFLFVIRKDDIILFSGKVSCP
Note: The complete sequence may include tag sequence, target protein sequence, linker sequence and extra sequence that is translated with the protein sequence for the purpose(s) of secretion, stability, solubility, etc.
If the exact amino acid sequence of this recombinant protein is critical to your application, please explicitly request the full and complete sequence of this protein before ordering.
蛋白標(biāo)簽：

N-terminal GST-tagged
產(chǎn)品提供形式：

Liquid or 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.
緩沖液：

If the delivery form is liquid, the default storage buffer is Tris/PBS-based buffer, 5%-50% glycerol. If the delivery form is lyophilized powder, the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose.
復(fù)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.
儲(chǔ)存條件：

Store at -20°C/-80°C upon receipt, aliquoting is necessary for mutiple use. Avoid repeated freeze-thaw cycles.
保質(zhì)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.
貨期：

3-7 business days
注意事項(xiàng)：

Repeated freezing and thawing is not recommended. Store working aliquots at 4°C for up to one week.
Datasheet & COA：

Please contact us to get it.

產(chǎn)品評(píng)價(jià)

靶點(diǎn)詳情

功能：

Might function as an inhibitor of Lys-specific proteases. Might influence the maturation of megakaryocytes via its action as a serpin.
基因功能參考文獻(xiàn)：
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
顯示更多

收起更多
相關(guān)疾?。?/div>
Keratoderma, palmoplantar, Nagashima type (PPKN)
亞細(xì)胞定位：

Cytoplasm.
蛋白家族：

Serpin family, Ov-serpin subfamily
組織特異性：

Predominantly expressed in mesangial cells. Expressed in the epidermis of the whole body.
數(shù)據(jù)庫(kù)鏈接：

HGNC: 13902

OMIM: 603357

KEGG: hsa:8710

STRING: 9606.ENSP00000337212

UniGene: Hs.138202