Thyroid cancer remains a rare disease in children less than age 10, with an annual incidence of less than one per million. It is more common in older children and adolescents, with 15.4 cases per million per year in 15-19 year olds. It has a peak incidence at age 50 and beyond.
Researchers at Queen's University have uncovered the actions of a mutated protein in cells linked to thyroid cancer.
Taranjit Gujral, lead author on the paper, developed three-dimensional models of the mutated RET protein implicated in a condition causing cancerous thyroid tumours.
The model allowed him to predict and compare the protein's molecular actions and to see that the protein was ten times more active than normal in cells associated with Multiple Endocrine Neoplasia 2B (MEN 2B) syndrome, an inherited cancer syndrome.
"We now know why this gene causes these tumours and can start looking at how best to target the mutant proteins so that the cells expressing them can be killed or stopped from growing," says Lois Mulligan, professor of pathology and molecular medicine with the Division of Cancer Biology and Genetics of the Queen's Cancer Research Institute.
MEN 2B is a dominantly inherited condition - the most severe of its kind - and is characterised by the early onset of thyroid tumours, sometimes even affecting infants, and can also cause developmental abnormalities including elongated bones, gastric problems and bumpy lips.
MEN 2B is currently treated with surgery, and other treatments, such as radiation and chemotherapy are not very effective. The study provides valuable tools for specific targeting of the actions of the protein that may aid in the development of anticancer therapies.
"The mutation may cause some new actions but it chiefly does some actions more efficiently than normal," said Mulligan.
The models created by Gujral, can be used further to help illuminate the actions of the protein with MEN 2B's other mutations.
The research team credits the transdisciplinary approach and its benefits for providing fresh perspectives in generating the new understanding of RET's role in MEN 2B.