EWSR1 Recombinant Monoclonal Antibody

說明書

中文名稱：

EWSR1 Recombinant Monoclonal Antibody
貨號：

CSB-RA959060A0HU
規格：

￥1320
圖片：
Flow cytometric analysis of EWS RNA binding protein 1 expression in HT-1080 cells using EWS RNA binding protein 1 antibody. Green, isotype control; red, EWS RNA binding protein 1.

Immunocytochemical staining of HT-1080 cells with EWS RNA binding protein 1 antibody. Nuclei were stained blue with DAPI; EWS RNA binding protein 1 was stained magenta with Alexa Fluor? 647. Images were taken using Leica stellaris 5. Protein abundance based on laser Intensity and smart gain: High. Scale bar: 20 μm.

Western blotting analysis using EWS RNA binding protein 1 antibody. Total cell lysates (30 μg) from various cell lines were loaded and separated by SDS-PAGE. The blot was incubated with EWS RNA binding protein 1 antibody and HRP-conjugated goat anti-rabbit secondary antibody respectively.
其他：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

Uniprot No.：

Q01844
基因名：

EWSR1
別名：

EWSR1; EWS RNA Binding Protein 1; EWS; Ewing Sarcoma Breakpoint Region 1; RNA-Binding Protein EWS; Ewings Sarcoma EWS-Fli1 (Type 1) Oncogene; Ewing Sarcoma Breakpoint Region 1 Protein; EWS Oncogene; BK984G1.4; EWS-FLI1
反應種屬：

Human, Mouse, Rat
免疫原：

Recombinant Human EWSR1 protein
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：

24C5
濃度：

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

Rabbit IgG in PBS (pH 7.4) containing 50% glycerol, and 0.02% sodium azide.
產品提供形式：

Liquid
應用范圍：

ELISA, WB, FC, ICC

推薦稀釋比：

Application	Recommended Dilution
WB	1:1000-1:5000
FC	1:200-1:2000
ICC	1:100-1:1000

Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Flow cytometry (FC) 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：

Might normally function as a transcriptional repressor. EWS-fusion-proteins (EFPS) may play a role in the tumorigenic process. They may disturb gene expression by mimicking, or interfering with the normal function of CTD-POLII within the transcription initiation complex. They may also contribute to an aberrant activation of the fusion protein target genes.
基因功能參考文獻：
1. ATG4B expression was observed markedly upregulated by EWS-FLI1 overexpression, and silencing of ATG4B dramatically inhibits autophagy in Ewing sarcoma cells. PMID: 28902354
2. Studies demonstrate that the translocation-derived fusion protein EF (EWS-FLI1) misregulates numerous genes involved in metabolism suggesting that EF is a master regulator of metabolic reprogramming in Ewing sarcoma, diverting metabolites toward biosynthesis. PMID: 28720588
3. Together, the data demonstrate the critical requirement of GGAA-microsatellites as EWS/FLI activating response elements in vivo and reveal an unexpected role for the EWS portion of the EWS/FLI fusion in binding to sweet-spot GGAA-microsatellites. PMID: 28847958
4. EWSR1 fusion protein is required for PAX7 expression in Ewing sarcoma and identify a candidate EWSR1-FLI1-bound PAX7 enhancer that coincides with both a consensus GGAA repeat-containing binding site and a peak of regulatory H3K27 acetylation. PMID: 28643791
5. In the 3 cases of the primary extraskeletal myxoid chondrosarcoma (EMC) of bone we found the most frequent and specific chromosomal translocation t(9:22) EWSR1-NR4A3 of the extraskeletal counterpart. PMID: 28249774
6. a breakpoint in the EWSR1-ATF1 fusion to be the same as that reported in HCCC. Established CCOC-T cells grew extremely slowly, but the cells showed highly invasive activity PMID: 28559020
7. study proposed that EWSR1 rearrangement was present in a subset of myoepithelial tumors of salivary glands, with variable morphological features PMID: 28648935
8. EWSR1 Gene Rearrangement is associated with Endometrial Primitive Neuroectodermal Tumor. PMID: 28801375
9. -cell lymphoblastic leukemia with t(11;22)(q24;q12) and EWSR1 rearrangement. PMID: 28104675
10. Kinetics of EWSR1 fusion sequence copy numbers in the plasma are correlated with changes of the tumor volume in patients with localized and metastatic disease. PMID: 27283964
11. Authors found that FUS, EWS and TAF15 expression is differentially regulated during brain development, both in time and in space. In particular, this study identifies a fine-tuned regulation of FUS and EWS during neuronal differentiation. PMID: 28453628
12. Recent advances in biologic and genomic understanding of these two cancers has expanded the potential for therapeutic advancement and prevention. In Ewing sarcoma, directed focus on inhibition of EWSR1-FLI1 and its effectors has produced promising results. PMID: 28561686
13. data suggest EWS/FLI binds to "promoter-like" and "enhancer-like" microsatellites to mediate activation and repression of target genes through different regulatory mechanisms PMID: 29091716
14. As spontaneous fluctuations in EWS-FLI1 levels of Ewing sarcoma cells in vitro and in vivo, associated with a switch between a proliferative, non-migratory EWS-FLI1-high and a non-proliferative highly migratory EWS-FLI1-low state, were recently described, our data provide a mechanistic basis for the underlying EWS-FLI1-dependent reversible cytoskeletal reprogramming of Ewing sarcoma cells. PMID: 28671673
15. Fusion of short fragments of EWSR1 to FLI1 is sufficient to recapitulate BAF complex retargeting and EWS-FLI1 activities; studies thus demonstrate that the physical properties of prion-like domains can retarget critical chromatin regulatory complexes to establish and maintain oncogenic gene expression programs. PMID: 28844694
16. Case Reports: maxillary sinus clear cell carcinomas with EWSR1-ATF1 gene fusion. PMID: 27916624
17. Results expand the spectrum of tumor types harboring EWSR1/FUS-ATF1 gene fusions to include a subgroup of conventional epithelioid malignant mesothelioma. PMID: 28505004
18. papillary thyroid carcinomas with EWSR1 rearrangement disclosed a lower percentage of nuclei with EWSR1 polysomy than those without EWSR1 rearrangement PMID: 28236059
19. The net result of combined lurbinectedin and irinotecan treatment was a complete reversal of EWS-FLI1 activity and elimination of established tumors in 30% to 70% of mice after only 11 days of therapy. Our results illustrate the preclinical safety and efficacy of a disease-specific therapy targeting the central oncogenic driver in Ewing sarcoma. PMID: 27697767
20. Aggregation of FET proteins FUS, EWSR1, and TAF15 mediate a pathological change in amyotrophic lateral sclerosis. (Review) PMID: 27311318
21. A subgroup of MAML2 fusion-negative mucoepidermoid carcinomas are actually clear cell carcinoma of the salivary gland with EWSR1 translocation. PMID: 27769871
22. Study found consistent DNA hypomethylation at enhancers regulated by the disease-defining EWS-FLI1 fusion protein, thus establishing epigenomic enhancer reprogramming as a ubiquitous and characteristic feature of Ewing sarcoma. PMID: 28134926
23. Report a distinct group of myxoid mesenchymal neoplasms occurring in children or young adults with a predilection for intracranial locations with EWSR1-AFT1/CREB1/CREM fusions. PMID: 28009602
24. We conclude that in the context of 22q11-12 regional alterations present in SMARCB1-deleted tumors, simultaneous EWSR1 involvement may be misinterpreted as equivalent to EWSR1 rearrangement. A detailed clinicopathologic correlation and supplementing the EWSR1 FISH assay with complementary methodology is mandatory for correct diagnosis PMID: 27218413
25. While our study confirmed that fluorescence in-situ hybridization is a sensitive and specific tool in the diagnosis of EWSR1-associated tumours, atypical fluorescence in-situ hybridization signals and classical rearrangement in entities other than EWSR1-associated tumours can occur PMID: 27385661
26. EWSR1 regulates the acetylation of microtubules in a cell cycle-dependent manner through its dynamic location on spindle MTs, and may be a novel regulator for mitosis progress independent of its translocation. PMID: 27341063
27. Studies provide evidence that FUS/TLS, EWS and TAF15 proteins play a major role in neurodegenerative disorders. (review). PMID: 27415968
28. We identified EWSR1 rearrangement in 25% of Ectomesenchymal chondromyxoid tumour PMID: 27010880
29. EWSR1-related rearrangement was detected extraskeletal myxoid chondrosarcoma PMID: 27402218
30. a novel EWSR1/ETS chimeric gene, was identified in a patient diagnosed with refractory AML, suggesting a potential role of leukemogenesis in rare cases of AML. This fusion gene is very likely to exhibit oncogenic potential by interfering with the p53/p21-dependent pathway. PMID: 27627705
31. LRWD1 is a novel regulator of EWS-FLI1 driven cell proliferation in Ewing sarcoma cells. EWS-FLI1 regulates LRWD1 expression and LRWD1 may contribute to EWS-FLI1 mediated transcriptional repression. PMID: 27760381
32. Ewing sarcoma may be susceptible to treatment with epigenetic inhibitors blocking BRD3/4 activity and the associated pathognomonic EWS-FLT1 transcriptional program. PMID: 26623725
33. FUS and EWS target genes involved in pathways at the RNA regulatory level PMID: 26573619
34. Identify SP1 and PI3K/AKT signaling as modulators of EWS/FLI1 gene expression in tumor cell lines. PMID: 26336820
35. Case Reports: 2 girls with primary renal myoepithelial carcinomas with a novel EWSR1-KLF15 fusion. PMID: 26523541
36. 3'-UTR poly(T/U) repeat of EWSR1 is altered in microsatellite unstable colorectal cancer. PMID: 25930744
37. study investigated EWSR1 status in ovarian hemangiomas; all cases were negative for EWSR1 rearrangement; 2 cases demonstrated additional intact copies of EWSR1 indicating aneusomy 22 or structural abnormality of chromosome 22 resulting in apparent duplication of the EWSR1 gene region PMID: 25851709
38. The paper investigates the presence of EWS/FLI-1 fusion in clinically diagnosed sarcoma belonging to different non-Ewing connective tissue tumors. PMID: 25914746
39. Results show that RNA-binding protein EWS binds and regulates CCDC6 expression at RNA and protein levels. PMID: 25751255
40. KLF17 is also a rare gene fusion partner to EWSR1-rearranged myoepithelial tumors. PMID: 25706482
41. The impairment of EWS-dependent midzone formation via the recruitment of Aurora B is a potential mechanism of Ewing sarcoma development. PMID: 25483190
42. Results show that although the cosegregation of the EWSR1 p.R471C substitution in the index family could not be established in people suffering from essential tremor, the EWSR1 p.R471C substitution is a candidate variant that needs to be further screened. PMID: 25375143
43. A key function of EWS-FLI1 in tumorigenesis is to maintain the epigenetic memory of activated homeobox genes programs. PMID: 25625846
44. Data suggest that aberrant cell cycle activation in Ewing sarcoma is due to the de-repression of transcription factor E2F targets of transcriptional induction and physical recruitment of E2F3 by fusion protein EWS-FLI1 replacing E2F4 on their promoters. PMID: 25712098
45. EWS/FLI-induced repression of alpha5 integrin and zyxin expression promotes tumor progression by supporting anchorage-independent cell growth. PMID: 25057021
46. These experiments establish systemic alternative splicing as an oncogenic process modulated by EWS-FLI1. PMID: 25737553
47. Case Reports: genetically confirmed primary renal sclerosing epithelioid fibrosarcoma with EWSR1-CREB3L1 gene fusion. PMID: 25353281
48. Describe EWSR1 gene rearrangement in a subset of myoepithelial carcinomas arising in minor and major salivary glands. PMID: 25581728
49. EWSR1-ATF1 fusion gene was found in hyalinizing clear cell carcinoma PMID: 25359601
50. EWSR1 rearrangement is associated with ewing sarcomas. PMID: 24293381
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相關疾病：

Ewing sarcoma (ES); Angiomatoid fibrous histiocytoma (AFH)
亞細胞定位：

Nucleus. Cytoplasm. Cell membrane. Note=Relocates from cytoplasm to ribosomes upon PTK2B/FAK2 activation.
蛋白家族：

RRM TET family
組織特異性：

Ubiquitous.
數據庫鏈接：

HGNC: 3508

OMIM: 133450

KEGG: hsa:2130

STRING: 9606.ENSP00000400142

UniGene: Hs.374477

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