Ewing Sarcoma Protein : A Key Player in Human Cancer

Author

Paronetto, Maria Paola

Source

International Journal of Cell Biology

Issue

Vol. 2013, Issue 2013 (31 Dec. 2013), pp.1-12, 12 p.

Publisher

Hindawi Publishing Corporation

Publication Date

2013-09-03

Country of Publication

Egypt

No. of Pages

12

Main Subjects

Biology

Abstract EN

The Ewing sarcoma protein (EWS) is a well-known player in cancer biology for the specific translocations occurring in sarcomas.

The EWS-FLI1 gene fusion is the prototypical translocation that encodes the aberrant, chimeric transcription factor, which is a landmark of Ewing tumors.

In all described Ewing sarcoma oncogenes, the EWS RNA binding domains are completely missing; thus RNA binding properties are not retained in the hybrid proteins.

However, it is currently unknown whether the absence of EWS function in RNA metabolism plays a role in oncogenic transformation or if EWS plays a role by itself in cancer development besides its contribution to the translocation.

In this regard, recent reports have highlighted an essential role for EWS in the regulation of DNA damage response (DDR), a process that counteracts genome stability and is often deregulated in cancer cells.

The first part of this review will describe the structural features of EWS and its multiple roles in the regulation of gene expression, which are exerted by coordinating different steps in the synthesis and processing of pre-mRNAs.

The second part will examine the role of EWS in the regulation of DDR- and cancer-related genes, with potential implications in cancer therapies.

Finally, recent advances on the involvement of EWS in neuromuscular disorders will be discussed.

Collectively, the information reviewed herein highlights the broad role of EWS in bridging different cellular processes and underlines the contribution of EWS to genome stability and proper cell-cycle progression in higher eukaryotic cells.

American Psychological Association (APA)

Paronetto, Maria Paola. 2013. Ewing Sarcoma Protein : A Key Player in Human Cancer. International Journal of Cell Biology،Vol. 2013, no. 2013, pp.1-12.
https://search.emarefa.net/detail/BIM-487632

Modern Language Association (MLA)

Paronetto, Maria Paola. Ewing Sarcoma Protein : A Key Player in Human Cancer. International Journal of Cell Biology No. 2013 (2013), pp.1-12.
https://search.emarefa.net/detail/BIM-487632

American Medical Association (AMA)

Paronetto, Maria Paola. Ewing Sarcoma Protein : A Key Player in Human Cancer. International Journal of Cell Biology. 2013. Vol. 2013, no. 2013, pp.1-12.
https://search.emarefa.net/detail/BIM-487632

Data Type

Journal Articles

Language

English

Notes

Includes bibliographical references

Record ID

BIM-487632