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CSTB Recombinant Monoclonal Antibody

  • 货号：
    CSB-RA914307A0HU
  • 规格：
    ￥1320
  • 图片：
    • Immunofluorescence staining of Hela cell with CSB-RA914307A0HU at 1:50, counter-stained with DAPI. The cells were fixed in 4% formaldehyde and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 591-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
  • 其他：

产品详情

  • Uniprot No.：
    P04080
  • 基因名：
    CSTB
  • 别名：
    Cystatin-B (CPI-B) (Liver thiol proteinase inhibitor) (Stefin-B), CSTB, CST6 STFB
  • 反应种属：
    Human
  • 免疫原：
    A synthesized peptide derived from human CSTB
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Monoclonal
  • 抗体亚型：
    Rabbit IgG
  • 纯化方式：
    Affinity-chromatography
  • 克隆号：
    11H4
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IF
  • 推荐稀释比：
    Application Recommended Dilution
    IF 1:50-1:200
  • 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：
    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