Researchers showed that a protein called JARID2 is necessary for the progression of rhabdomyosarcomas – cancers that resemble developing muscles and affect up to 60 children in the UK each year.
Like many paediatric cancers, rhabdomyosarcomas are often caused by genetic defects, in this case produced after two genes called PAX3 and FOXO1 have been aberrantly joined together.
The team found that the protein built by this PAX3-FOXO1 fusion gene switches on JARID2, which stops the cancer cells being able to turn into muscle tissue and causes them to proliferate without control.
Investigating how JARID2 achieves this effect, they discovered that it can affect the way that DNA is packaged up in a structure called chromatin. These changes alter the pattern of expression of several genes which together control how the cells grow and differentiate into specialised muscle cells.
Dr Janet Shipley, Team Leader in Sarcoma Molecular Pathology, said: "When we blocked the gene which produces JARID2 in rhabdomyosarcoma cells, the rate of cell division returned to more normal levels, and the cells began to develop the characteristics of healthy muscle cells, unlike in tumours where they remain undifferentiated."
The research is at an early stage, but Dr Shipley is cautiously optimistic. "We have so far only looked at controlling JARID2 in cells using laboratory techniques. However, the results suggest that if we can find a way to switch off this or similar molecules in the cancers of patients, we might have a better chance of improving the outlook for children with this potentially devastating cancer."
The research is published in the journal Oncogene.