A recent study published in Molecular
Cancer Research has shown a promising new development in the fight against Diffuse Intrinsic Pontine Glioma (DIPG), a rare but lethal brain tumour in children. Radiation is currently the only approved therapy for DIPG patients, and that only succeeds in decreasing symptoms rather than curing the tumour, so any development in the fight against this devastating disease is encouraging.
The study has found that a ‘tumour suppressor’ gene - p16 - is turned off by a histone
mutation (H3.3K26M), which is found in up to 70 per cent of DIPG patients. The
study therefore suggests that turning p16
back on in these patients may be an effective therapeutic approach, and has
tested this hypothesis in an early laboratory study.
Histones are essentially a DNA spool; they help package a large DNA strand into the tiny nucleus of each living cell. These histones also regulate which genes turn on and off, and when histones are mutated, this process can go catastrophically wrong.
When p16 is
repressed, the study demonstrated that cells divided faster and led to tumour
growth. This mutation combined with an overactive growth factor (PDGF), further
accelerated brain stem tumours formation.
The researchers involved in this study found that p16 could be turned back on using a drug
that prevents DNA methylation (a process that controls gene expression). This
drug restored p16 function, which
slowed down tumour growth.
The study will continue in the lab before progressing to human patients. As founding members of the DIPG Collaborative, we are excited about the growing knowledge base around DIPG and other associated paediatric brain tumours, which we are hopeful will lead to more effective therapies being developed quickly.
You can read more quotes from the researchers involved in this study here.
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