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Ewing sarcoma breakpoint region 1

EWS, EWSR1
This gene encodes a multifunctional protein that is involved in various cellular processes, including gene expression, cell signaling, and RNA processing and transport. The protein includes an N-terminal transcriptional activation domain and a C-terminal RNA-binding domain. Chromosomal translocations between this gene and various genes encoding transcription factors result in the production of chimeric proteins that are involved in tumorigenesis. These chimeric proteins usually consist of the N-terminal transcriptional activation domain of this protein fused to the C-terminal DNA-binding domain of the transcription factor protein. Mutations in this gene, specifically a t(11;22)(q24;q12) translocation, are known to cause Ewing sarcoma as well as neuroectodermal and various other tumors. Alternative splicing of this gene results in multiple transcript variants. Related pseudogenes have been identified on chromosomes 1 and 14. [provided by RefSeq, Jul 2009] (from NCBI)
Top mentioned proteins: FLI-1, CAN, HAD, CD99, POLYMERASE
Papers on EWS
Utility of CD99 Paranuclear Expression in the Differential Diagnosis of Merkel Cell Carcinoma.
New
Lino-Silva et al., Mexico. In Int J Surg Pathol, Feb 2016
MATERIAL AND METHODS: We explore paranuclear dot-like CD99 expression in several small, round blue cell neoplasms, including neuroendocrine neoplasms, Ewing sarcomas/primitive neuroectodermal tumors (EWS/PNET), melanomas, small cell lung carcinomas (SCC), lymphoblastic lymphoma/leukemia, and rhabdomyosarcomas, in comparison with 33 cases of MCC, to determine the specificity of the paranuclear dot-like CD99 expression in MCC.
Advances in the Clinicopathologic and Molecular Classification of Cutaneous Mesenchymal Neoplasms.
Review
New
Doyle et al., Dublin, Ireland. In Histopathology, Feb 2016
Genetic characterization of several soft tissue tumour types that occur in the skin has resulted in the identification of diagnostically useful markers: ALK gene rearrangement with corresponding ALK protein expression by immunohistochemistry in epithelioid fibrous histiocytoma; WWTR1-CAMTA1 fusion gene with CAMTA1 protein expression in epithelioid haemangioendothelioma; MYC amplification and overexpression in radiation-associated angiosarcoma; and EWSR1 gene rearrangement in cutaneous myoepithelial tumours.
Functional Genomic Screening Reveals Splicing of the EWS-FLI1 Fusion Transcript as a Vulnerability in Ewing Sarcoma.
New
Caplen et al., Nashville, United States. In Cell Rep, Feb 2016
We show that Ewing sarcoma cells harboring a genomic breakpoint that retains exon 8 of EWSR1 require the RNA-binding protein HNRNPH1 to express in-frame EWS-FLI1.
Novel BCOR-MAML3 and ZC3H7B-BCOR Gene Fusions in Undifferentiated Small Blue Round Cell Sarcomas.
New
Antonescu et al., Birmingham, United Kingdom. In Am J Surg Pathol, Feb 2016
About two-thirds of EWSR1-negative SBRCTs are associated with CIC-DUX4-related fusions, whereas another small subset shows BCOR-CCNB3 X-chromosomal paracentric inversion.
Ewing sarcoma: The clinical relevance of the insulin-like growth factor 1 and the poly-ADP-ribose-polymerase pathway.
Review
New
Gelderblom et al., Leiden, Netherlands. In Eur J Cancer, Feb 2016
IGF-1R: The IGF-1R pathway is known to be deregulated by the EWSR1-FLI1 translocation which makes it a potential target for therapy.
The drug situation in Europe: an overview of data available on illicit drugs and new psychoactive substances from European monitoring in 2015.
Review
New
Simon et al., Lisbon, Portugal. In Addiction, Jan 2016
METHODS: The European reporting system formally covers all 28 European Union (EU) Member States, Norway and Turkey and incorporates multiple indicators alongside an early warning system (EWS) on uncontrolled new psychoactive substances (NPS).
Tigroid background in cytology of hyalinizing clear cell carcinoma of the salivary gland.
New
Hsieh et al., Taipei, Taiwan. In Diagn Cytopathol, Jan 2016
UNASSIGNED: Hyalinizing clear cell carcinoma (HCCC) is a rare and low grade salivary gland carcinoma with a unique recurrent EWSR1 gene translocation.
Ewing Sarcoma: Current Management and Future Approaches Through Collaboration.
Review
New
Impact
Oberlin et al., Villejuif, France. In J Clin Oncol, Oct 2015
The many insights into the biology of the EWS-FLI1 protein in the initiation and progression of ES remain to be translated into novel therapeutic strategies.
Linking germline and somatic variation in Ewing sarcoma.
New
Impact
Davis et al., Chapel Hill, United States. In Nat Genet, Sep 2015
A new study links a germline variant to Ewing sarcoma disease susceptibility and EWSR1-FLI1-mediated gene activation.
Chimeric EWSR1-FLI1 regulates the Ewing sarcoma susceptibility gene EGR2 via a GGAA microsatellite.
New
Impact
Delattre et al., Paris, France. In Nat Genet, Sep 2015
Ewing sarcoma is characterized by fusions between EWSR1 and members of the ETS gene family, usually EWSR1-FLI1, leading to the generation of oncogenic transcription factors that bind DNA at GGAA motifs.
microRNA and Bone Cancer.
Review
Nugent, Dublin, Ireland. In Adv Exp Med Biol, 2014
The EWS-FLI1 fusion protein produced in Ewing sarcoma has been shown to induce changes in miRNA expression.
Primary sclerosing epithelioid fibrosarcoma of kidney with variant histomorphologic features: report of 2 cases and review of the literature.
Review
Aki et al., Ankara, Turkey. In Diagn Pathol, 2014
Fluorescence in situ hybridization assay using dual staining probes detected EWSR1-CREB3L1 fusion in each lesion, which is characteristic molecular findings of SEF.
Biochemical Properties and Biological Functions of FET Proteins.
Review
Impact
Parker et al., Boulder, United States. In Annu Rev Biochem, 2014
Members of the FET protein family, consisting of FUS, EWSR1, and TAF15, bind to RNA and contribute to the control of transcription, RNA processing, and the cytoplasmic fates of messenger RNAs in metazoa.
ChIP-ping away at EWS/ETS transcription networks.
Impact
Denny, Los Angeles, United States. In Cancer Cell, 2014
In this issue of Cancer Cell, Riggi and colleagues use a genomic approach to define two distinct molecular mechanisms through which the chimeric EWS/FLI1 oncoprotein regulates target genes in Ewing sarcoma, expanding a framework upon which to model the target gene network and test strategies for antagonizing growth of this tumor.
Targeting the EWSR1-FLI1 oncogene-induced protein kinase PKC-β abolishes ewing sarcoma growth.
GeneRIF
Tirode et al., Paris, France. In Cancer Res, 2012
We found that transcriptional activation of PRKCB was directly regulated by the chimeric fusion oncogene EWSR1-FLI1 that drives ewing sarcoma growth.
Impairment of p53 acetylation by EWS-Fli1 chimeric protein in Ewing family tumors.
GeneRIF
Zhu et al., Shenyang, China. In Cancer Lett, 2012
Data indicate EWS-Fli1 might deacetylate p53 to inhibit its transcriptional function and protein stability via the recruitment of HDAC1. Results elucidate a novel molecular mechanism about the abrogation of p53 pathway by EWS-Fli1 in EFTs pathogenesis.
Tumors with EWSR1-CREB1 and EWSR1-ATF1 fusions: the current status.
Review
GeneRIF
Fisher et al., London, United Kingdom. In Am J Surg Pathol, 2012
Review: discuss clinicopathologic and molecular features of group of neoplasms unified by the presence of EWSR1-CREB1 and EWSR1-ATF1 genetic fusions.
NR4A3 rearrangement reliably distinguishes between the clinicopathologically overlapping entities myoepithelial carcinoma of soft tissue and cellular extraskeletal myxoid chondrosarcoma.
GeneRIF
Mentzel et al., Nijmegen, Netherlands. In Virchows Arch, 2012
three of nine (33 %) myoepithelial carcinomas of soft tissue and four of five (80 %) cellular extraskeletal myxoid chondrosarcomas showed an EWSR1 rearrangement.
Malignant small round blue cell tumor of the kidney without EWSR1 rearrangement: report of a case and review of the literature.
Review
GeneRIF
Pagliaro et al., Houston, United States. In Clin Genitourin Cancer, 2012
EWSR1 rearrangement is not associated with malignant small round blue cell tumor of the kidney.
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