EWSR1 (Ewing Sarcoma Breakpoint Region 1 Protein, EWS Oncogene, RNA-binding Protein EWS, EWS, bK984G1.4, CAGF29)

Ewing sarcoma breakpoint region 1 is a protein belonging to the family of RNA binding proteins which contain a single RRM domain. It also has a single Zinc Finger domain of the ZnF_RBZ subtype found in Ran binding proteins. Ran is a small G protein related to p21-Ras which regulates the import and export of proteins to the nucleus. EWSR1 appears to be a multifunctional protein that is involved in various cellular processes, including gene expression, cell signaling, and RNA processing and transport. In particular, EWSR1 is required for cell survival in the central nervous system (1). The EWSR1 gene was identified based on its location at the chromosome 22 breakpoint of the t(11;22)(q24;q12) translocation that characterizes Ewing sarcoma and related neuroectodermal tumors. This results in the production of an aberrant fusion protein containing the N-terminus of a protein encoded by the Friend leukemia virus integration 1 (FLI1) protein and the C-terminal of the EWS protein. The chromosomal region harboring the EWS gene appears to be particularly prone to translocations, and such translocations can result in the production of several other aberrant fusion proteins, in each case with C-terminus of EWS fused with an another protein (2, see also the OMIM link). EWSR1 is ubiquitely expressed and is localized primarily in the nucleus of cell (3). It has also been found in the cytoplasm, and associated with the plasma membrane in a fashion regulated by the protein kinase Pyk2 (4). Expression of EWS in the various subcellular compartments is affected by the methylation state of its RNA-binding domain (5). Localization of EWS in different subcellular compartments reflects a dynamic distribution during cell cycle. EWS associates with and stablizes microtubules, leading to cell cycle arrest (6). EWSR1, TAF15 and FUS/TLS are closely related proteins which are believed to have evolved by gene multiplication, and all have been implication in the etiology of some forms of Amyotrophic Lateral Sclerosis (ALS, a.k.a. Lou Gehrig's Disease) (7). The HGNC name for EWS is EWSR1.\n\nApplications:\nSuitable for use in Immunocytochemistry, Immunofluorescence and Western Blot. Other applications not tested.\n\nRecommended Dilution:\nImmunofluorescence: 1:500\nWestern Blot: 1:1000\nOptimal dilutions to be determined by the researcher.\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

031357

Size

100ul

Applications

ICC, IF, WB

Hosts

Mouse

Reactivities

Hum

Form

Supplied as a liquid in PBS, 0.05% sodium azide.

P Type

Mab

Purity

Purified by Protein G affinity chromatography.

Isotype

IgG2b,k

References

1. Azuma M, Embree LJ, Sabaawy H, Hickstein DD. Ewing sarcoma protein ewsr1 maintains mitotic integrity and proneural cell survival in the zebrafish embryo. PLoS One. 2:e979 (2007).\n\n2. Delattre O, Zucman J, Plougastel B, Desmaze C, Melot T, Peter M, Kovar H, Joubert I, de Jong P, Rouleau G, et al. Gene fusion with an ETS DNA-binding domain caused by chromosome translocation in human tumours. Nature. 359:162-5 (1992).\n\n3. Andersson MK, St

Additional Info

Recognizes EWS specifically both in Western Blots and in immunocytochemical experiments. On blots reveals a prominent 85kD band, and on cells in tissue culture the antibody stains mainly the nucleus.