How transcription and RNA processing drive Ewing sarcoma
Official title: Dysregulated transcription processes in Ewing sarcoma
Funded at Research Inst Nationwide Children's Hosp · NIH-11330324 · Data updated 2026-11-12
Researchers are looking at how a fusion gene causes abnormal RNA transcription and splicing in Ewing sarcoma in children and young adults.
Quick facts
| Grant type | R01 grant |
|---|---|
| Study type | NIH-funded research |
| Funding institution | Research Inst Nationwide Children's Hosp NIH-funded |
| Lab location | 1 site (Columbus, United States) |
| Project start | May 1, 2020 |
| Project end | April 30, 2027 |
| Funding year | 2025 |
| Award (this year) | $291,262 |
| NIH project number | 7R01CA241554-06 |
| Project ID | NIH-11330324 on NIH RePORTER |
Who could benefit from this research
✓Could help
Children and young adults diagnosed with Ewing sarcoma could potentially contribute tumor samples or be candidates for future trials based on this research.
✕Less likely to help
People without Ewing sarcoma or those needing immediate clinical therapy should not expect direct, short-term benefits from this laboratory-focused research.
What this research studies
From my perspective as a patient or family member, the team studies how the EWSR1-FLI1 fusion makes RNA polymerase II overly active and leads to toxic RNA structures called R-loops that disrupt normal RNA splicing. They use lab work on tumor cells and genomic RNAi screens to find which RNA processing steps EwS cells are most dependent on. The researchers also study proteins like CDK7, CDK9, and DHX9 that interact with the fusion to find molecular weak points. The goal is to reveal targets that could be used for new treatments or to guide future clinical trials.
Why it matters
Potential benefit: If successful, this work could point to new targeted treatments or biomarkers that improve outcomes and reduce side effects for people with Ewing sarcoma.
How similar studies have gone: Prior laboratory studies, including the team's earlier Nature report linking the fusion to hyperactive transcription and targets like CDK7/CDK9, support the approach, but translating these findings into effective patient treatments remains early-stage.
Where this research is happening
-
Research Inst Nationwide Children's Hosp
Columbus, United States Active
Researchers
- Principal investigator: Bishop, Alexander James — Research Inst Nationwide Children's Hosp
- Study coordinator: Bishop, Alexander James
Related research
Other NIH-funded projects on the same topics.
-
AI-powered prognostic model for Ewing sarcoma using digital pathology
Researchers are building an AI tool to predict survival for children and adults with Ewing sarcoma using digitized pathology slides.
-
RAPID: A patient-centered, fast-tracked drug trial platform for young people with relapsed Ewing sarcoma and DSRCT
A patient-friendly, open-label phase 2 platform trial tests promising drug combinations for teens and young adults whose Ewing sarcoma or DSRCT has come back or not responded to treatment.
-
TGFβ brakes immune evasion and metastasis in Ewing sarcoma during radiation
A physician-scientist will study whether blocking TGFβ during radiation can help CAR T cells fight Ewing sarcoma and improve patient outcomes.
-
Function and Targeting of ETV6 in Ewing Sarcoma
A scientist at Case Western Reserve University is studying a specific protein, ETV6, that helps a rare bone cancer called Ewing sarcoma, with the goal of finding targeted therapies that could spare healthy cells.
-
Targeting immune markers in Ewing Sarcoma to guide safer, more effective therapies
A funded project at Stanford aims to use immune markers on Ewing Sarcoma to develop targeted immunotherapies that work in children and adults with this cancer.
-
Ewing sarcoma: how DNA repair weaknesses affect risk and treatment
A clinician-researcher team at Dana-Farber is studying how DNA damage repair weaknesses help drive Ewing sarcoma in kids and teens, with the goal of better predicting risk and guiding therapy.
About this research
- This is an active NIH-funded research project — typically early-stage science, not a clinical trial accepting patient enrollment.
- Some NIH-funded labs run parallel clinical studies or seek volunteers for related work. To check, contact the principal investigator or institution listed above.
- For full project details, budget, and progress reports, visit the official NIH RePORTER page.