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EWSR1 Antibody

  • 货号:
    CSB-PA007875GA01HU
  • 规格:
    ¥3,900
  • 其他:

产品详情

  • Uniprot No.:
    Q01844
  • 基因名:
    EWSR1
  • 别名:
    bK984G1.4 antibody; bK984G1.4 Ewing sarcoma breakpoint region 1 protein antibody; Ewing sarcoma breakpoint region 1 antibody; Ewing sarcoma breakpoint region 1 protein antibody; Ewings sarcoma EWS Fli1 type 1 oncogene antibody; EWS antibody; EWS oncogene antibody; EWS RNA binding protein 1 antibody; EWS_HUMAN antibody; EWSR 1 antibody; Ewsr1 antibody; EWSR1 protein antibody; RNA binding protein EWS antibody; RNA-binding protein EWS antibody
  • 宿主:
    Rabbit
  • 反应种属:
    Human,Mouse,Rat
  • 免疫原:
    Human EWS
  • 免疫原种属:
    Homo sapiens (Human)
  • 抗体亚型:
    IgG
  • 纯化方式:
    Antigen Affinity purified
  • 浓度:
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液:
    PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
  • 产品提供形式:
    Liquid
  • 应用范围:
    ELISA,WB
  • 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:
    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.
  • 基因功能参考文献:
    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. s 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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  • 相关疾病:
    Ewing sarcoma (ES); Angiomatoid fibrous histiocytoma (AFH)
  • 亚细胞定位:
    Nucleus. Cytoplasm. Cell membrane. Note=Relocates from cytoplasm to ribosomes upon PTK2B/FAK2 activation.
  • 蛋白家族:
    RRM TET family
  • 组织特异性:
    Ubiquitous.
  • 数据库链接:

    HGNC: 3508

    OMIM: 133450

    KEGG: hsa:2130

    STRING: 9606.ENSP00000400142

    UniGene: Hs.374477