CSTB Recombinant Monoclonal Antibody

說明書

中文名稱：

CSTB重組抗體
貨號：

CSB-RA914307A0HU
規格：

￥1320
圖片：
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：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品描述：

CSTB Recombinant Monoclonal Antibody（CSB-RA914307A0HU）是針對胱抑素B（Cystatin B）靶點開發的高特異性科研工具，適用于探索該蛋白在溶酶體蛋白酶調控及神經退行性疾病中的分子機制。本抗體通過重組表達技術制備，經ELISA和免疫熒光（IF）雙重驗證，在IF實驗中展現出優異的亞細胞定位性能，推薦使用稀釋比例為1:50-1:200。實驗數據顯示，該抗體能特異性識別內源性CSTB蛋白，在Hela細胞的免疫熒光實驗中清晰呈現核周區域信號分布，與靶蛋白的生物學定位特征一致，且不同批次間檢測結果穩定可靠。適用于研究CSTB在細胞應激反應、溶酶體穩定性調控以及癲癇肌陣攣（如EPM1）相關疾病模型中的功能解析，可支持蛋白質相互作用研究、疾病相關信號通路分析及特定細胞類型的蛋白表達水平檢測。該產品為研究者提供精準的靶標檢測方案，滿足基礎科研中蛋白定性、定量及定位分析需求，尤其適用于神經生物學、細胞自噬等領域的高分辨率實驗體系。
Uniprot No.：

P04080
基因名：

CSTB
別名：

Cystatin-B (CPI-B) (Liver thiol proteinase inhibitor) (Stefin-B), CSTB, CST6 STFB
反應種屬：

Human
免疫原：

A synthesized peptide derived from human CSTB
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

11H4
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
產品提供形式：

Liquid
應用范圍：

ELISA, IF

推薦稀釋比：

Application	Recommended Dilution
IF	1:50-1:200

Protocols：

ELISA Protocol
Immunofluorescence (IF) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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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