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  4. Recombinant Human Cystatin-B (CSTB)

Recombinant Human Cystatin-B (CSTB)

In Stock
  • 货号：
    CSB-EP006100HU
  • 规格：
    ￥1344
  • 图片：
    • (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
  • 其他：

产品详情

  • 纯度：
    Greater than 85% as determined by SDS-PAGE.
  • 基因名：
    CSTB
  • Uniprot No.：
  • 别名：
    CHROW21; CPI B; CPI-B; CST 6; CST6; CSTB; Cystatin B (stefin B); Cystatin B; Cystatin-B; CYTB; CYTB_HUMAN; EPM1; EPM1A; Liver thiol proteinase inhibitor; PME; Stefin-B; STF B; STFB; ULD
  • 种属：
    Homo sapiens (Human)
  • 蛋白长度：
    Full Length
  • 来源：
    E.coli
  • 分子量：
    27.1kDa
  • 表达区域：
    1-98aa
  • 氨基酸序列
    MMCGAPSATQPATAETQHIADQVRSQLEEKENKKFPVFKAVSFKSQVVAGTNYFIKVHVGDEDFVHLRVFQSLPHENKPLTLSNYQTNKAKHDELTYF
    Note: The complete sequence including tag sequence, target protein sequence and linker sequence could be provided upon request.
  • 蛋白标签：
    N-terminal 6xHis-SUMO-tagged
  • 产品提供形式：
    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.
  • 缓冲液：
    Tris-based buffer,50% glycerol
  • 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：
    3-7 business days
  • 注意事项：
    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.

产品评价

靶点详情

  • 功能：
    This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B.
  • 基因功能参考文献：
    1. cystatin B expression was significantly and inversely correlated with lung tumor stage and tumor grade PMID: 29037838
    2. The results demonstrate that cystatin B interferes with the STAT-1 signaling and IFN-beta-antiviral responses perpetuating HIV in macrophage reservoirs. PMID: 27137788
    3. apoptosis is accompanied by degradation of the cysteine cathepsin inhibitor stefin B (StfB). CatD did not exhibit a crucial role in this step. However, this degradation was partially prevented through pre-incubation with the antioxidant N-acetyl cysteine PMID: 28543404
    4. Homozygous for a c.218dupT (p.His75Serfs*2) mutation in exon 3 of CSTB causes neurodegeneration, progressive cerebral volume loss and diffuse hypomyelination. PMID: 28378817
    5. CSTB downregulation may promote the development of gastric cancer. PMID: 28281969
    6. It was shown that decreased expression of cystatin B enhances cathepsin activity in Niemann-Pick C cerebellar degeneration patient fibroblasts. PMID: 26908626
    7. High expression of stefin B may be an important factor contributing to the development and metastasis of Hepatocellular Carcinoma. PMID: 26753874
    8. CSTB null mutation associated with microcephaly, early developmental delay, and severe dyskinesia. PMID: 26843564
    9. Data shows that CYTB and ANXA4 overexpression may be involved in carcinogenesis and histopathological differentiation of ovarian clear cell carcinoma and suggest they may serve as a potential diagnostic biomarkers. PMID: 25633807
    10. A role for disease-causing mutations in cystatin B gene in patients with juvenile myoclonic epilepsy was not supported. PMID: 25752200
    11. Even though the majority of EPM1 patients have a uniform genetic mutation, the actual size of the longer CSTB expansion mutation allele is likely to have a modulating effect on the age at disease onset, myoclonus severity, and cortical neurophysiology. PMID: 25770194
    12. The study shows detection of stefin B dimers in HEK293 cells and the importance of their residual activity. PMID: 25047918
    13. glutamate dehydrogenase is a euchromatin-associated enzyme, and its H3 clipping activity is regulated by chromatin structure, histone modifications and an in vivo inhibitor. PMID: 25263734
    14. detected a homozygous expansion of dodecamer repeats in the CSTB gene in four patients with clinical diagnosis of ULD. PMID: 23883076
    15. The increased CSTB expression in ovarian tissue represents tumor progression and is dysregulated by the TGF-beta signaling pathway. PMID: 24452274
    16. A reciprocal influence of CSTB and SOD1 at the gene expression level and for a direct interaction of the two proteins, is reported. PMID: 24234043
    17. The present study was performed on two more missense mutants of human stefin B, G50E and Q71P, and they similarly showed numerous aggregates upon overexpression. PMID: 24909779
    18. The co-localization of stefin B wild type and EPM1 mutants with cathepsins showed that cathepsins accumulate around the aggregates formed by the EPM1 mutants. PMID: 23362198
    19. Skull thickening and an increased prevalence of abnormal findings in skeletal radiographs of patients with EPM1 suggest that this condition is connected to defective cystatin B function. PMID: 23010349
    20. This study suggested that CSTB mutations other than the common dodecamer expansion predict particular phenotypes, including marked seizure severity and polymorphous seizure types. PMID: 23205931
    21. Elevated StefA mRNA level is associated with invasive glioblastoma. PMID: 22287159
    22. S-glutathionylation and S-cysteinylation were described as extensive PTM of a salivary protein and the first time that these PTMs were detected in naturally occurring cystatin B. PMID: 22057043
    23. patients compound heterozygous for the dodecamer repeat expansion and the c.202C>T mutations seem to have a severer form of Unverricht-Lundborg disease (EPM1) than patients homozygous for the expansion mutation PMID: 21757863
    24. At pH 7.0 the mutant H75W folded in three kinetic phases to a native-like intermediate, analogous to folding of stefin B at pH 4.8. PMID: 22033403
    25. Intracellular stefin b aggregation shows a negative correlation with cell survival PMID: 20078424
    26. Stefin B interacts with histones and cathepsin L in the nucleus PMID: 20075068
    27. oligomers of stefin B and amyloid-beta interact in vitro and in cells PMID: 19955183
    28. Oligonucleotides containing EPM1 repeat adopt secondary structures that may facilitate strand slippage thereby causing the expansion. PMID: 11697734
    29. Intramolecular i-motif structure at acidic pH for progressive myoclonus epilepsy (EPM1) repeat d(CCCCGCCCCGCG)n. PMID: 11697735
    30. analysed eight markers flanking CSTB(GT10-D21S1890-D21S1885-D21S2040-D21S1259- CSTB-D21S1912-PFKL-D21S171) and one intragenic variant in the CSTB 3' UTR (A2575G) PMID: 12215838
    31. first demonstration of cysteine protease activity being regulated by CSTB activity in a biological context; effects of decreased CSTB activity in EPM1 pathogenesis may be mediated by cathepsins through increased activity of cathepsins S and L PMID: 12452481
    32. Prefibrillar oligomers/aggregates of stefin B also increase the surface pressure at an air-water interface, i.e. they have amphipathic character and are surface seeking. PMID: 15955063
    33. These data show that cystatin B inhibits bone resorption by down-regulating intracellular cathepsin K activity despite increased osteoclast survival. PMID: 16321512
    34. Study shows that copper binding by stefin B inhibits the amyloid fibril formation and, to a lesser degree, the initial aggregation. PMID: 16939620
    35. Several alternatively spliced CSTB isoforms were identified in patients with progressive myoclonus epilepsy of Unverricht-Lundborg type . PMID: 17003839
    36. Results describe the influence of pH and trifluoroethanol on amyloid fibril growth and morphology from human stefin B. PMID: 17701471
    37. cystatin B in vivo has a polymeric structure sensitive to the redox environment and that overexpression of the protein generates aggregates. PMID: 17920138
    38. CSTB is specifically overexpressed in most HCCs and is also elevated in the serum of a large proportion of HCC patients PMID: 18281540
    39. Data show that wild-type stefin B and its Y31 isoform are able to form pores in planar lipid bilayers, whereas the G4R isoform destroys the bilayer by a non pore-forming process. PMID: 18397316
    40. The mechanism of amyloid-fibril formation by stefin B: temperature and protein concentration dependence of the rates;the observed kinetics follow the nucleation and growth behavior observed for many other amyloidogenic proteins. PMID: 18636508
    41. potential role for CSTB in HIV replication in placental macrophages PMID: 18951626
    42. cystatin B interacts with STAT-1 and the levels of STAT-1 tyrosine phosphorylation (but not serine phosphorylation) between uninfected and HIV-infected PM and MDM are differentially regulated. PMID: 19342095

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  • 相关疾病：
    Epilepsy, progressive myoclonic 1 (EPM1)
  • 亚细胞定位：
    Cytoplasm. Nucleus.
  • 蛋白家族：
    Cystatin family
  • 数据库链接：

    HGNC: 2482

    OMIM: 254800

    KEGG: hsa:1476

    STRING: 9606.ENSP00000291568

    UniGene: Hs.695