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Identifying new treatments for children with rhabdomyosarcoma

Every year in the UK around 70 children are diagnosed with rhabdomyosarcoma, a cancer that resembles developing skeletal muscle. The available treatments have remained largely unchanged for 20 years and, although they do work for some children, can cause long-term side effects that seriously affect their normal development. New treatments are needed so that more children can recover and lead a normal life.

Over the last 20 years or so there has been very little change in the treatment regime for young patients with rhabdomyosarcoma. We’re looking for more effective, targeted therapy with fewer long term side effects.

Project Progress...

Project Details

  • Project Title

    Enhancer of Zest Homolog 2 (EZH2) as a therapeutic target in Rhabdomyosarcomas

  • Lead Researcher

    Dr Zoë Walters

  • Research Centre

    Institute of Cancer Research

  • City & Institution Postcode

    Surrey, SM2 5NG

  • Start Date

    1 January 2015

  • Duration

    5 years

  • Grant Amount

    --

Overview

Rhabdomyosarcoma (RMS) is rare, with only about 70 children in the UK diagnosed each year. But it’s also hard to treat. The treatments available for it haven’t changed much in two decades, and, although they’re effective for some children, they often have side effects which affect their normal development. We’re looking at whether new drugs can help treat RMS more effectively and with less impact on the children.

Sometimes we can find more of certain proteins in tumours than in healthy tissues, which can give us a clue about what to target with new drugs. Dr Zoë has already identified a protein called EZH2 that is present at higher levels in tumour tissue. During this project, she’s going to look more at EZH2 to find out whether drugging it will stop and potentially cure RMS.

What difference will this project make?

During the project, Dr Zoë will determine how much the protein EZH2 is overexpressed in RMS and whether by drugging the protein we can stop and potentially kill these tumours. EZH2 (whose full name is Enhancer of Zeste Homolog 2) can be found in higher levels in the tumour tissues, and we already know that some newly developed drugs can stop it working.

Dr Zoë will perform more tests on EZH2 and these drugs in laboratory testing, and she hopes to provide evidence to incorporate these drugs into clinical testing.

If successful, this new, targeted and effective treatment for children affected by RMS may eventually prolong lives and reduce the side effects of treatment.

About the Research Team

Dr Zoë became one of our first Research Fellows in 2014. Her PhD was at the University of Sussex, working on genes and proteins involved in the development of the fruit fly wing. By studying them in normal development using model organisms we see how these proteins work, and how they lead to tumour formation when things go wrong.

After her first postdoc at Cardiff University she joined Janet Shipley’s lab at the Institute of Cancer Research (ICR) in 2008. Since then she’s been working on a number of proteins that contribute to the growth of RMS tumours, and has unique skills for studying how these proteins may be exploited as therapeutic targets for treating Rhabdomyosarcoma.

“My interest in science can be traced all the way back to the age of seven when I drew a picture of myself in a white ‘dress’, which turned out to be a lab coat. It had the caption – when I grow up I want to be a researcher – ! I have always been passionate about the study of genetics and how that relates to biology, with a particular focus on childhood cancer research. I hope that one day my work will lead to a new treatment for this disease to improve survival rates and reduce the damaging side effects for the patients.”

Dr Zoë Walters

Zoe's golden moment for September Childhood Cancer Awareness Month

As part of September Childhood Cancer Awareness Month, we asked Zoe to tell us about a golden moment in her research. Zoe talks about her project that looks into Identifying new treatments for children with rhabdomyosarcoma, her funding from Children with Cancer UK, her golden moment and a message to families and children undergoing childhood cancer treatment.

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