Mutations linked to aggressive childhood brain tumours
January 30, 2012 in NEWS by Suzanne Elvidge
Diffuse intrinsic pontine glioma (DIPG) is a rare and lethal childhood brain cancer, and researchers taking part in the Pediatric Cancer Genome Project (PCGP) have found a link with a gene not previously connected with cancer, in a paper published in Nature Genetics.
Source: Steve Spicer www.steve-spicer.com
DIPG, rarely seen in adults, is fatal in 90% of children within two years and accounts for 10-15% of tumours of the brain and central nervous system in children. The researchers carried out whole-genome sequencing of tumours and found that 78% of DIPG samples shows mutations in the gene H3F3A coding for histone H3.3 or the gene HIST1H3B, coding for histone H3.1. Histones help DNA fold so that it can fit into the nucleus.
“We are hopeful that identifying these mutations will lead us to new selective therapeutic targets, which are particularly important since this tumour cannot be treated surgically and still lacks effective therapies,” said Suzanne Baker, Ph.D., co-author and co-leader of the St. Jude Neurobiology and Brain Tumor Program.
Knowledge of these mutations could help create diagnostics and therapeutics for DIPG, and these mutations may also play a role in other aggressive paediatric brain tumours.
Other news to come out of the genome project includes a gene linked to the aggressive childhood eye cancer retinoblastoma, and potential new approaches to leukaemia.
Wu, G., Broniscer, A., McEachron, T., Lu, C., Paugh, B., Becksfort, J., Qu, C., Ding, L., Huether, R., Parker, M., Zhang, J., Gajjar, A., Dyer, M., Mullighan, C., Gilbertson, R., Mardis, E., Wilson, R., Downing, J., Ellison, D., Zhang, J., & Baker, S. (2012). Somatic histone H3 alterations in pediatric diffuse intrinsic pontine gliomas and non-brainstem glioblastomas Nature Genetics DOI: 10.1038/ng.1102
