Universidad de Murcia. Departamento de Biología Celular e Histología
Doi
Abstract
Despite our deep understanding of
neuroblastic tumors, some patients still suffer treatment
failure, so pre-treatment risk stratification still requires
improvement and the search for new therapeutic targets
must continue. Here we correlated prognostic clinical
and biological features of neuroblastic tumors with the
density of extracellular matrix glycosaminoglycans (the
main components of the extracellular matrix ‘ground
substance’), in nearly 400 primary samples. We also
studied the relationship between the density of
extracellular matrix glycosaminoglycans and the
expression of B3GALT6, an enzyme required for their
synthesis. We associated a decrease in glycosaminoglycans with neuroblastomas that were
histopathologically poorly-differentiated or undifferentiated, as well as with metastatic disease, and 1p36
deleted tumors. This decrease in glycosaminoglycans
was also related to abnormal nuclear B3GALT6
expression in neuroblastic cells. These findings point
towards the importance of the ground substance in the
aggressiveness of neuroblastic tumors, which should
therefore be considered when developing novel therapies
for treating neuroblastomas