 Summary Information
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Location: 22q12
Alias: EWS
Overview:
The EWS (EWSR1) gene is involved in translocations in Ewing's sarcoma, clear cell sarcoma, desmoplastic small round cell tumor and myxoid liposarcoma. The Ewing sarcoma family of tumours is characterised by recurrent translocations that fuse EWS to one of
the following genes FLI1 (>90% of cases), ERG, ETV1, E1AF and FEV. In clear cell sarcoma, DSRCT and myxoid liposarcoma EWS is fused to ATF1, WT1 and CHOP respectively.
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| Gene Database Entries for EWSR1
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OMIM
GeneCard (Weizmann Institute)
Atlas of Genetics and Cytogenetics in Oncology and Haematology
Swiss-Prot
Locus Link
UniGene
GenAtlas
GDB
Nomenclature (search for EWSR1)
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| Other Related Resources
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 Ewing's Sarcoma Resources
FLI1 (11q24.1-q24.3)
ERG (21q22.3)
Medline Search: EWSR1 (PubMed)
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Medline Search: EWS-FLI1 (PubMed)
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- Medline Search: cancer AND gene AND (EWSR1[TI] OR EWS[TI]) (PubMed)
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| t(11;22)(q24;q12) EWS-FLI1 Translocation in Ewing's Sarcoma
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The t(11;22)(q24;q12) translocation is present in over 90% of Ewing's sarcoma cases. The resulting EWS-FLI1 fusion gene has been demonstrated to have oncogenic potential. Many alternative forms of the translocation exist, corresponding to variations in the
locations of the EWS and FLI1 breakpoints. The most common form, "Type 1", accounts for approximately 60% of cases and consists of the first seven exons of EWS joined to exons 6-9 of FLI1. "Type 2", accounts for approximately 25% of cases and also includes
FLI1 exon 5. The type of translocation has been related to prognosis.
Medline Search: Ewing's sarcoma AND chromosome 11 AND cheomosome 22 (PubMed)
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Ewing's Sarcoma Genetics
Ewing's Sarcoma : Clinical and Epidemiological Information
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| EWS-FLI1 and the Ewing's Family of Tumours
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Delattre O. et al. The Ewing family of tumors--a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med 1994; 331:294-9 Related articles (PubMed)
Medline Search: Ewing's sarcoma AND chromosome 11 AND cheomosome 22 (PubMed)
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Ewing's Sarcoma Genetics
Ewing's Sarcoma : Clinical and Epidemiological Information
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| EWS-FLI1 Transcript Detection by PCR
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Delattre O. et al. The Ewing family of tumors--a subgroup of small-round-cell tumors defined by specific chimeric transcripts. N Engl J Med 1994; 331:294-9 Related articles (PubMed)
Medline Search: Ewing's sarcoma AND polymerase chain reaction (PubMed)
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Ewing's Sarcoma Genetics
Ewing's Sarcoma : Clinical and Epidemiological Information
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| EWS-FLI1 Fusion Transcript Structure and Prognosis
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Lin PP, et al. Differential transactivation by alternative EWS-FLI1 fusion proteins correlates with clinical heterogeneity in Ewing's sarcoma. Cancer Res. 1999 Apr 1;59(7):1428-32 Related articles (PubMed)
de Alava E. et al. EWS-FLI1 fusion transcript structure is an independent determinant of prognosis in Ewing's sarcoma. J Clin Oncol 1998; 16:1248-55 Related articles (PubMed)
Zoubek A. et al. Does expression of different EWS chimeric transcripts define clinically distinct risk groups of Ewing tumor patients? J Clin Oncol 1996; 14:1245-51 Related articles (PubMed)
Medline Search: Ewing's sarcoma AND (EWS-FLI1 OR 11;22) AND (transcript OR type) (PubMed)
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Ewing's Sarcoma : Clinical and Epidemiological Information
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| Antisense EWS-FLI1
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Dohjima T, et al. Preferential down-regulation of phospholipase C-beta in Ewing's sarcoma cells transfected with antisense EWS-Fli-1. Br J Cancer 2000;82(1):16-9 Related articles (PubMed)
Tanaka K, et al. EWS-Fli1 antisense oligodeoxynucleotide inhibits proliferation of human Ewing's sarcoma and primitive neuroectodermal tumor cells. J Clin Invest 1997 Jan 15;99(2):239-47 Related articles (PubMed)
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Ewing's Sarcoma Genetics
Ewing's Sarcoma : Clinical and Epidemiological Information
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| t(21;22) EWS-ERG Translocations in Ewing's Sarcoma
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Sorensen PH, et al. A second Ewing's sarcoma translocation, t(21;22), fuses the EWS gene to another ETS-family transcription factor, ERG Nat Genet. 1994; 6:146-51 Related articles (PubMed)
Ida K. et al. EWS-FLI-1 and EWS-ERG chimeric mRNAs in Ewing's sarcoma and primitive neuroectodermal tumor. Int J Cancer 1995; 63:500-4 Related articles (PubMed)
Kaneko Y. et al. EWS-ERG fusion transcript produced by chromosomal insertion in a Ewing sarcoma. Genes Chromosomes Cancer. 1997; 18:228-31 Related articles (PubMed)
Medline Search: Ewing's sarcoma AND (chromosome 21 AND chromosome 22) OR (EWS[title] AND ERG[tit (PubMed)
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Ewing's Sarcoma Genetics
Ewing's Sarcoma : Clinical and Epidemiological Information
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| t(17;22)(q12;q12) EWS-E1AF Translocation in Ewing's Sarcoma
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Ishida S, et al. The genomic breakpoint and chimeric transcripts in the EWSR1-ETV4/E1AF gene fusion in Ewing sarcoma. Cytogenet Cell Genet. 1998;82(3-4):278-83. Related articles (PubMed)
Urano F, et al. Molecular analysis of Ewing's sarcoma: another fusion gene, EWS-E1AF, available for diagnosis. Jpn J Cancer Res 1998 Jul;89(7):703-11 Related articles (PubMed)
Kaneko Y, et al. Fusion of an ETS-family gene, EIAF, to EWS by t(17;22)(q12;q12) chromosome translocation in an undifferentiated sarcoma of infancy. Genes Chromosomes Cancer 1996 Feb;15(2):115-21 Related articles (PubMed)
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Ewing's Sarcoma Genetics
Ewing's Sarcoma : Clinical and Epidemiological Information
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| t(7;22)(p22;q12) EWS-ETV1 Translocation in Ewing's Sarcoma
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Im YH, et al. EWS-FLI1, EWS-ERG, and EWS-ETV1 oncoproteins of Ewing tumor family all suppress transcription of transforming growth factor beta type II receptor gene. Cancer Res 2000 Mar 15;60(6):1536-40 Related articles (PubMed)
Jeon IS, et al. A variant Ewing's sarcoma translocation (7;22) fuses the EWS gene to the ETS gene ETV1. Oncogene. 1995 Mar 16;10(6):1229-34 Related articles (PubMed)
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Ewing's Sarcoma Genetics
Ewing's Sarcoma : Clinical and Epidemiological Information
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| t(9;22)(q22;q12) in Extraskeletal Myxoid Chondrosarcoma
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Sciot R, et al. t(9;22)(q22-31;q11-12) is a consistent marker of extraskeletal myxoid chondrosarcoma: evaluation of three cases. Mod Pathol 1995 Sep;8(7):765-8 Related articles (PubMed)
Stenman G, et al. Translocation t(9;22)(q22;q12) is a primary cytogenetic abnormality in extraskeletal myxoid chondrosarcoma. Int J Cancer 1995 Aug 9;62(4):398-402 Related articles (PubMed)
Hirabayashi Y, et al. Translocation (9;22)(q22;q12). A recurrent chromosome abnormality in extraskeletal myxoid chondrosarcoma. Cancer Genet Cytogenet 1995 May;81(1):33-7 Related articles (PubMed)
Hinrichs SH, et al. Myxoid chondrosarcoma with a translocation involving chromosomes 9 and 22 Cancer Genet Cytogenet 1985 Jan 15;14(3-4):219-26 Related articles (PubMed)
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Chondrosarcoma Genetics
Chondrosarcoma : Clinical and Epidemiological Information
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| t(11;22) EWS-WT1 in Desmoplastic Small Round Cell Tumour
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Lee SB, et al. The EWS-WT1 translocation product induces PDGFA in desmoplastic small round-cell tumour. Nat Genet 1997; 17(3):309-13 Related articles (PubMed)
Gerald WL, et al. Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants. J Clin Oncol. 1998; 16(9):3028-36 Related articles (PubMed)
Medline Search: T1 AND EWS AND desmoplastic small round cell tumour (PubMed)
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| t(12; 22)(q13; q12) Translocation in Clear Cell Sarcoma
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The t(12;22)(q13;q12) is characteristic of malignant melanoma of soft parts (clear cell sarcoma). This fuses the ATF1 gene on chromosome 12 with the EWS gene on chromosome 22.
Lung RW, et al. The cellular oncogene EWS/activating transcription factor 1 is unable to activate adenovirus-borne promoters: implications for cytotoxic prodrug therapy of malignant melanoma of soft parts. Cancer Gene Ther 2000 Mar;7(3):396-406 Related articles (PubMed)
Bosilevac JM, et al. Tumor cell viability in clear cell sarcoma requires DNA binding activity of the EWS/ATF1 fusion protein. J Biol Chem 1999 Dec 3;274(49):34811-8 Related articles (PubMed)
Pellin A, et al. New type of chimeric fusion product between the EWS and ATFI genes in clear cell sarcoma (malignant melanoma of soft parts). Genes Chromosomes Cancer 1998 Dec;23(4):358-60 Related articles (PubMed)
Speleman F, et al. Malignant melanoma of the soft parts (clear-cell sarcoma): confirmation of EWS and ATF-1 gene fusion caused by a t(12;22) translocation. Mod Pathol 1997 May;10(5):496-9 Related articles (PubMed)
Fujimura Y, et al. The EWS-ATF-1 gene involved in malignant melanoma of soft parts with t(12;22) chromosome translocation, encodes a constitutive transcriptional activator. Oncogene 1996 Jan 4;12(1):159-67 Related articles (PubMed)
Zucman J, et al. EWS and ATF-1 gene fusion induced by t(12;22) translocation in malignant melanoma of soft parts. Nat Genet 1993 Aug;4(4):341-5 Related articles (PubMed)
Bridge JA, et al. Chromosomal abnormalities in clear cell sarcoma. Implications for histogenesis. Am J Clin Pathol 1990 Jan;93(1):26-31 Related articles (PubMed)
Medline Search: clear cell sarcoma AND (atf1 OR atf-1) (PubMed)
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