Study
Targeting the DNA Repair Pathway in Ewing Sarcoma
Study ID | Alternative Stable ID | Type |
---|---|---|
EGAS00001000839 | Other |
Study Description
Ewing’s Sarcoma is a bone and soft tissue tumor that primarily affects adolescents and young adults. With current therapies, 70% of patients with localized disease survive but survival for metastatic and recurrent disease is poor. Whole genome sequencing of 19 Ewing’s Sarcoma tumors showed that STAG2 was mutated in 10% (2/19) of the tumors and STAG2 protein was absent in 14% (13/106) tumors by immunohistochemical staining. Previous studies have shown that glioblastoma cells lacking STAG2 are more sensitive to poly-ADP ribose polymerase (PARP) inhibitors. We found that Ewing’s Sarcoma cell lines are sensitive to PARP inhibitors irrespective of STAG2 protein expression. Ewing Sarcoma cell lines are defective in double strand DNA break repair. PARP inhibitor cytotoxicity in Ewing’s Sarcoma cells was potentiated 10-1,000 fold by DNA damaging agents (irinotecan and temozolomide). To extend these studies in vivo, we developed an orthotopic Ewing’s Sarcoma mouse model and performed pharmacokinetic and pharmacodynamic studies with three different PARP inhibitors (BMN-673, olaparib ... (Show More)
Study Datasets 3 datasets.
Click on a Dataset ID in the table below to learn more, and to find out who to contact about access to these data
Dataset ID | Description | Technology | Samples |
---|---|---|---|
EGAD00001001020 |
DATA FILES FOR SJEWS-WGS
|
Illumina HiSeq 2000 | 38 |
EGAD00001001432 |
PCGP Germline Study Whole Genome Sequencing
|
Illumina HiSeq 2000 | 1337 |
EGAD00001001433 |
PCGP Germline Study Whole Exome Sequencing
|
Illumina HiSeq 2000 | 906 |
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