XL EWSR1 BA

Break Apart Probe

Order Number
D-6011-100-OG
Package Size
100 µl
Labels
  
Chromosome
22

Description

XL EWSR1 BA

XL EWSR1 BA is designed as a break apart probe. The orange labeled probe hybridizes proximal to the breakpoint in the EWSR1 gene region at 22q12, the green labeled probe hybridizes distal to the breakpoint.

This probe is intended for methanol/acetic-acid fixed cells and tissue sections.

Clinical Details

The Ewing sarcoma (EWS) is a rare and highly aggressive cancer with an incidence of about three in one millions Caucasians. The incidence in Africans is significantly lower. The EWS is more common among children and young adults than in adults and mostly arises in bones and to lower extend in soft tissue. EWS is typified by chromosomal translocations resulting in fusion genes between the EWS RNA Binding Protein 1 (EWSR1) and a member of the group of ETS transcription factors. t(11;22)(q24;q12) is the most common of these translocations represented by the EWSR1-FLI1 fusion gene with a frequency of about 85%. The EWSR1 part contributes a strong transcriptional domain while FLI1 is providing the ETS family DNA binding domain. The chimeric protein is dysregulating target genes leading to oncogenic transformation and is absolutely required for tumorigenesis in EWS. Other translocation partners are known, but no difference in survival has been observed between different fusion genes. Although complex karyotypes are rare in EWS, gain of chromosome 1q, 8, 12 and loss of 9p21 (CDKN2A) and 16q is observed. The EWSR gene is expressed in several tissues and is involved in other tumor disease. EWSR1 has manifold functions and is involved in different control mechanisms in the cell. EWS is genetically stable and complex karyotypes are rare.

Clinical Applications

  • Solid Tumors (Solid Tumors)

Images

XL EWSR1 BA

XL EWSR1 BA hybridized to lymphocytes. Interphases with two fusion signals each are displayed.

Expected Patterns

Expected Pattern 1

Normal Cell:
Two green-orange colocalization/fusion signals (2GO).

Expected Pattern 2

Aberrant Cell (typical results):
One green-orange colocalization/fusion signal (1GO), one separate green (1G) and orange (1O) signal each resulting from a chromosome break in the respective locus.

Literature

  • Delattre et al (1992) Nature 359:162-165
  • Smith et al (2006) Cancer Cell 9:405-416
  • Sannino et al (2017) Future Oncol. 13:1207-1211

Downloads

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