CSTB Antibody
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货号:CSB-PA006100GA01HU
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规格:¥3,900
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其他:
产品详情
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Uniprot No.:P04080
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基因名:CSTB
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别名:CHROW21 antibody; CPI B antibody; CPI-B antibody; CST 6 antibody; CST6 antibody; CSTB antibody; Cystatin B (stefin B) antibody; Cystatin B antibody; Cystatin-B antibody; CYTB antibody; CYTB_HUMAN antibody; EPM1 antibody; EPM1A antibody; Liver thiol proteinase inhibitor antibody; PME antibody; Stefin-B antibody; STF B antibody; STFB antibody; ULD antibody
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宿主:Rabbit
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反应种属:Human
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免疫原:Human CSTB
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免疫原种属:Homo sapiens (Human)
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抗体亚型:IgG
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纯化方式:Antigen Affinity Purified
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浓度:It differs from different batches. Please contact us to confirm it.
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保存缓冲液:PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
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产品提供形式:Liquid
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应用范围:ELISA,WB,IHC
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Protocols:
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储存条件:Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
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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.
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靶点详情
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功能:This is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B.
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基因功能参考文献:
- cystatin B expression was significantly and inversely correlated with lung tumor stage and tumor grade PMID: 29037838
- The results demonstrate that cystatin B interferes with the STAT-1 signaling and IFN-beta-antiviral responses perpetuating HIV in macrophage reservoirs. PMID: 27137788
- 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
- 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
- CSTB downregulation may promote the development of gastric cancer. PMID: 28281969
- It was shown that decreased expression of cystatin B enhances cathepsin activity in Niemann-Pick C cerebellar degeneration patient fibroblasts. PMID: 26908626
- High expression of stefin B may be an important factor contributing to the development and metastasis of Hepatocellular Carcinoma. PMID: 26753874
- CSTB null mutation associated with microcephaly, early developmental delay, and severe dyskinesia. PMID: 26843564
- 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
- A role for disease-causing mutations in cystatin B gene in patients with juvenile myoclonic epilepsy was not supported. PMID: 25752200
- 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
- The study shows detection of stefin B dimers in HEK293 cells and the importance of their residual activity. PMID: 25047918
- 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
- detected a homozygous expansion of dodecamer repeats in the CSTB gene in four patients with clinical diagnosis of ULD. PMID: 23883076
- The increased CSTB expression in ovarian tissue represents tumor progression and is dysregulated by the TGF-beta signaling pathway. PMID: 24452274
- 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
- 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
- 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
- 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
- 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
- Elevated StefA mRNA level is associated with invasive glioblastoma. PMID: 22287159
- 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
- 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
- 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
- Intracellular stefin b aggregation shows a negative correlation with cell survival PMID: 20078424
- Stefin B interacts with histones and cathepsin L in the nucleus PMID: 20075068
- oligomers of stefin B and amyloid-beta interact in vitro and in cells PMID: 19955183
- Oligonucleotides containing EPM1 repeat adopt secondary structures that may facilitate strand slippage thereby causing the expansion. PMID: 11697734
- Intramolecular i-motif structure at acidic pH for progressive myoclonus epilepsy (EPM1) repeat d(CCCCGCCCCGCG)n. PMID: 11697735
- 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
- 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
- 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
- These data show that cystatin B inhibits bone resorption by down-regulating intracellular cathepsin K activity despite increased osteoclast survival. PMID: 16321512
- Study shows that copper binding by stefin B inhibits the amyloid fibril formation and, to a lesser degree, the initial aggregation. PMID: 16939620
- Several alternatively spliced CSTB isoforms were identified in patients with progressive myoclonus epilepsy of Unverricht-Lundborg type . PMID: 17003839
- Results describe the influence of pH and trifluoroethanol on amyloid fibril growth and morphology from human stefin B. PMID: 17701471
- cystatin B in vivo has a polymeric structure sensitive to the redox environment and that overexpression of the protein generates aggregates. PMID: 17920138
- CSTB is specifically overexpressed in most HCCs and is also elevated in the serum of a large proportion of HCC patients PMID: 18281540
- 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
- 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
- potential role for CSTB in HIV replication in placental macrophages PMID: 18951626
- 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)
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亚细胞定位:Cytoplasm. Nucleus.
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蛋白家族:Cystatin family
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数据库链接:
HGNC: 2482
OMIM: 254800
KEGG: hsa:1476
STRING: 9606.ENSP00000291568
UniGene: Hs.695
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