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Ewing sarcoma breakpoint region 1

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Title: Ewing sarcoma breakpoint region 1  
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Subject: Myxoid liposarcoma, FUS, ERG (gene), Chromosomal abnormalities, 46, XX/XY
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Ewing sarcoma breakpoint region 1

EWS RNA-binding protein 1

PDB rendering based on 2cpe.
Available structures
PDB Ortholog search: PDBe, RCSB
Symbols  ; EWS; bK984G1.4
External IDs GeneCards:
RNA expression pattern
Species Human Mouse
RefSeq (mRNA)
RefSeq (protein)
Location (UCSC)
PubMed search

RNA-binding protein EWS is a protein that in humans is encoded by the EWSR1 gene on human chromosome 22.[1][2] This gene encodes a putative RNA binding protein. Alternative splicing of this gene results in two products.[2]

Clinical relevance

Mutations in this gene, specifically a t(11;22)(q24;q12) translocation, are known to cause Ewing sarcoma and other members of the Ewing family of tumors. Other mutations have also been associated to amyotrophic lateral sclerosis.[3]


Ewing sarcoma breakpoint region 1 has been shown to interact with POU4F1,[4] SF1,[5] BARD1,[6] Small nuclear ribonucleoprotein polypeptide C,[7] PTK2B[8] and Zinc finger protein 165.[9]ERG,[10] ATF-1,[11]


  1. ^ Delattre O, Zucman J, Plougastel B, Desmaze C, Melot T, Peter M, Kovar H, Joubert I, de Jong P (October 1992). "Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours". Nature 359 (6391): 162–5.  
  2. ^ a b "Entrez Gene: EWSR1 Ewing sarcoma breakpoint region 1". 
  3. ^ Couthouis, J.; Hart, M. P.; Erion, R.; King, O. D.; Diaz, Z.; Nakaya, T.; Ibrahim, F.; Kim, H.-J.; Mojsilovic-Petrovic, J.; Panossian, S.; Kim, C. E.; Frackelton, E. C.; Solski, J. A.; Williams, K. L.; Clay-Falcone, D.; Elman, L.; McCluskey, L.; Greene, R.; Hakonarson, H.; Kalb, R. G.; Lee, V. M. Y.; Trojanowski, J. Q.; Nicholson, G. A.; Blair, I. P.; Bonini, N. M.; Van Deerlin, V. M.; Mourelatos, Z.; Shorter, J.; Gitler, A. D. (27 March 2012). "Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis". Human Molecular Genetics.  
  4. ^ Thomas, G Ruth; Latchman David S (2002). "The pro-oncoprotein EWS (Ewing's Sarcoma protein) interacts with the Brn-3a POU transcription factor and inhibits its ability to activate transcription". Cancer Biol. Ther. (United States) 1 (4): 428–32.  
  5. ^ Zhang, D; Paley A J; Childs G (July 1998). "The transcriptional repressor ZFM1 interacts with and modulates the ability of EWS to activate transcription". J. Biol. Chem. (UNITED STATES) 273 (29): 18086–91.  
  6. ^ Spahn, Laura; Petermann Robert; Siligan Christine; Schmid Johannes A; Aryee Dave N T; Kovar Heinrich (August 2002). "Interaction of the EWS NH2 terminus with BARD1 links the Ewing's sarcoma gene to a common tumor suppressor pathway". Cancer Res. (United States) 62 (16): 4583–7.  
  7. ^ Knoop, L L; Baker S J (August 2000). "The splicing factor U1C represses EWS/FLI-mediated transactivation". J. Biol. Chem. (UNITED STATES) 275 (32): 24865–71.  
  8. ^ Felsch, J S; Lane W S; Peralta E G (May 1999). "Tyrosine kinase Pyk2 mediates G-protein-coupled receptor regulation of the Ewing sarcoma RNA-binding protein EWS". Curr. Biol. (ENGLAND) 9 (9): 485–8.  
  9. ^ Rual, Jean-François; Venkatesan Kavitha, Hao Tong, Hirozane-Kishikawa Tomoko, Dricot Amélie, Li Ning, Berriz Gabriel F, Gibbons Francis D, Dreze Matija, Ayivi-Guedehoussou Nono, Klitgord Niels, Simon Christophe, Boxem Mike, Milstein Stuart, Rosenberg Jennifer, Goldberg Debra S, Zhang Lan V, Wong Sharyl L, Franklin Giovanni, Li Siming, Albala Joanna S, Lim Janghoo, Fraughton Carlene, Llamosas Estelle, Cevik Sebiha, Bex Camille, Lamesch Philippe, Sikorski Robert S, Vandenhaute Jean, Zoghbi Huda Y, Smolyar Alex, Bosak Stephanie, Sequerra Reynaldo, Doucette-Stamm Lynn, Cusick Michael E, Hill David E, Roth Frederick P, Vidal Marc (October 2005). "Towards a proteome-scale map of the human protein-protein interaction network".  
  10. ^ Ohno T, Ouchida M, Lee L, Gatalica Z, Rao, VN and Reddy, ES (Oct 1994). "The EWS gene, involved in Ewing family of tumors, malignant melanoma of soft parts and desmoplastic small round cell tumors, codes for an RNA binding protein with novel regulatory domains". Oncogene 9 (10): 3087–97.  
  11. ^ Fujimura Y, Ohno T, Siddique H, Lee L, Rao VN, Reddy ES (January 1996). "The EWS-ATF-1 gene involved in malignant melanoma of soft parts with t(12;22) chromosome translocation, encodes a constitutive transcriptional activator". Oncogene 12 (1): 159–67.  

Further reading

External links

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