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Human Cystatin-B (CSTB/CST6/STFB) ELISA kit

  • 中文名稱:
    人胱抑素B(CSTB/CST6/STFB)酶聯免疫試劑盒
  • 貨號:
    CSB-E16835h
  • 規格:
    96T/48T
  • 價格:
    ¥3600/¥2500
  • 其他:

產品詳情

  • 產品描述:
    人胱抑素B(CSTB/CST6/STFB)酶聯免疫試劑盒(CSB-E16835h)為雙抗夾心法ELISA試劑盒,定量檢測血清、血漿、組織培養上清液、尿液、唾液、組織勻漿、細胞裂解物樣本中的CSTB含量。CSTB即半胱氨酸蛋白酶抑制劑B,是一種內源性的半胱氨酸蛋白酶抑制劑。其突變與進行性肌陣攣性癲癇1型相關。研究機制上,它主要通過抑制半胱氨酸蛋白酶活性,維持細胞內蛋白代謝平衡,在神經系統的穩定等方面發揮重要作用。試劑盒檢測范圍為0.313 ng/mL-20 ng/mL,靈敏度為0.233 ng/mL。該產品適用于基礎科研中探索胱抑素B在神經退行性疾病模型、腫瘤微環境調控、炎癥信號通路中的表達規律,或作為生物標志物在體液樣本中的濃度變化研究,為疾病機制解析及藥物開發提供實驗支持。本品僅用于科研,不用于臨床診斷,產品具體參數及操作步驟詳見產品說明書。
  • 別名:
    CHROW21 ELISA Kit; CPI B ELISA Kit; CPI-B ELISA Kit; CST 6 ELISA Kit; CST6 ELISA Kit; CSTB ELISA Kit; Cystatin B (stefin B) ELISA Kit; Cystatin B ELISA Kit; Cystatin-B ELISA Kit; CYTB ELISA Kit; CYTB_HUMAN ELISA Kit; EPM1 ELISA Kit; EPM1A ELISA Kit; Liver thiol proteinase inhibitor ELISA Kit; PME ELISA Kit; Stefin-B ELISA Kit; STF B ELISA Kit; STFB ELISA Kit; ULD ELISA Kit
  • 縮寫:
    CSTB
  • Uniprot No.:
  • 種屬:
    Homo sapiens (Human)
  • 樣本類型:
    serum, plasma, cell culture supernates, urine, saliva, tissue homogenates, cell lysates
  • 檢測范圍:
    0.313 ng/mL-20 ng/mL
  • 靈敏度:
    0.233 ng/mL
  • 反應時間:
    1-5h
  • 樣本體積:
    50-100ul
  • 檢測波長:
    450 nm
  • 研究領域:
    Others
  • 測定原理:
    quantitative
  • 測定方法:
    Sandwich
  • 精密度:
    Intra-assay Precision (Precision within an assay): CV%<8%        
    Three samples of known concentration were tested twenty times on one plate to assess.    
    Inter-assay Precision (Precision between assays): CV%<10%        
    Three samples of known concentration were tested in twenty assays to assess.      
                   
  • 線性度:
    To assess the linearity of the assay, samples were spiked with high concentrations of human CSTB/CST6/STFB in various matrices and diluted with the Sample Diluent to produce samples with values within the dynamic range of the assay.  
      Sample Serum(n=4)    
    1:1 Average % 104    
    Range % 101-106    
    1:2 Average % 91    
    Range % 88-97    
    1:4 Average % 96    
    Range % 91-104    
    1:8 Average % 89    
    Range % 84-94    
  • 回收率:
    The recovery of human CSTB/CST6/STFB spiked to levels throughout the range of the assay in various matrices was evaluated. Samples were diluted prior to assay as directed in the Sample Preparation section.  
     
    Sample Type Average % Recovery Range    
    Serum (n=5) 104 99-108    
    EDTA plasma (n=4) 98 93-105    
                   
                   
  • 標準曲線:
    These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.  
     
    ng/ml OD1 OD2 Average Corrected    
    20 2.411 2.588 2.500 2.340    
    10 1.702 1.811 1.757 1.597    
    5 1.166 1.163 1.165 1.005    
    2.5 0.654 0.699 0.677 0.517    
    1.25 0.423 0.411 0.417 0.257    
    0.625 0.266 0.255 0.261 0.101    
    0.313 0.203 0.211 0.207 0.047    
    0 0.155 0.164 0.160      
  • 數據處理:
  • 貨期:
    3-5 working days

產品評價

靶點詳情

  • 最新研究進展:
    CSTB是一種半胱氨酸蛋白酶抑制劑,主要在神經系統中發揮作用,調節神經元的生長和存活。最近的研究表明,CSTB還可以通過抑制胰島素受體信號通路來影響胰島素的信號轉導,從而調節血糖代謝和胰島素抵抗。
     
  • 功能:
    This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B.
  • 基因功能參考文獻:
    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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  • 相關疾病:
    Epilepsy, progressive myoclonic 1 (EPM1)
  • 亞細胞定位:
    Cytoplasm. Nucleus.
  • 蛋白家族:
    Cystatin family
  • 數據庫鏈接:

    HGNC: 2482

    OMIM: 254800

    KEGG: hsa:1476

    STRING: 9606.ENSP00000291568

    UniGene: Hs.695



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