Discovery Could Prevent Childhood Brain Tumor

Recent findings published in the journal Developmental Cell show that the progression of medulloblastoma, the most common brain tumour found in children, could be prevented in some cases.

As sonic Hedgehog belongs to a family of proteins that provide cells the information needed for the embryo to develop properly, researchers discovered that it also plays a significant role in tumorigenesis, the process that transforms normal cells into cancer cells.

"Our team studied a protein called Boc, which is a receptor located on the cell surface that detects Sonic Hedgehog," said Lukas Tamayo-Orrego, PhD student in Dr. Charron's laboratory and co-first author of the study, in a news release. "We had previously shown that Boc is important for the development of the cerebellum, the part of the brain where medulloblastoma arises, so we decided to further investigate its role."

"With this study, we found that the presence of Boc is required for Sonic Hedgehog to induce DNA damage," adds Dr. Charron, Director of the Molecular Biology of Neural Development research unit at the IRCM. "In fact, Boc causes DNA mutations in tumour cells, which promotes the progression of precancerous lesions into advanced medulloblastoma."

"Our study shows that when Boc is inactivated, the number of tumours is reduced by 66 per cent," says Frederic Mille, PhD, co-first author of the article and former postdoctoral fellow in Dr. Charron's research unit. "The inactivation of Boc therefore reduces the development of early medulloblastoma into advanced tumours."

The study authors found how medulloblastoma ranks among the leading cuases of cancer-related mortality in children. Yet current treatments for the health issue, such as radiation therapy and chemotherapy can prove very successful in relieving and even curing the problem, it can dramatically damage brain cells in infants and toddlers, resulting in long-term harm. 

"As a result, many children who undergo these treatments suffer serious side effects including cognitive impairment and disorders," concluded Dr. Charron. "Our results indicate that Boc could potentially be targeted to develop a new therapeutic approach that would stop the growth and progression of medulloblastoma and could reduce the adverse side effects of current treatments."