Expression of EAAT2 in Dysembryoplastic Neuroepithelial Tumors (DNT) and Angiocentric Gliomas (AGs) and its Possible Role in the Pathogenesis of the Epilepsy

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Dysembryoplastic neuroepithelial tumours (DNTs) are closely tied to the experience of seizures and epilepsy in children. Angiocentric glioma (AG) is a rare cerebral tumor that is also associated with both seizures and epilepsy. Better understanding the formation of both of these tumors could lead to advancements in the treatment of pediatric epilepsy. In both tumor types it is believed that the excitatory amino acid transporter 2 (EAAT2) plays a key role in the pathogenesis, or origin, of DNT and AG related seizures. EAAT2 is a protein that transports the molecule glutamate. Loss of EAAT2 causes glutamate accumulation, meaning there is much more glutamate than there would be normally. At normal concentrations, glutamate is necessary for the function of the brain. However, at heightened levels the cells of the brain can become over-excited. This consequent hyper-excitability is what researchers believe could be tied to the development of seizures and epilepsy. Previous studies have demonstrated a loss of EAAT2 in patients with DNTs and AGs, leading researchers to believe it might have therapeutic significance. Additionally, inducing an increased EAAT2 protein expression could serve as a potential therapeutic target for the treatment of DNT and AG related epilepsy. The access to the CBTN samples and the consequent possibility to obtain a critical number of rare AGs specimens, a tumor for which researchers have already demonstrated the loss of EAAT2, would be essential to confirm this data in a larger population and to compare the expression of EAAT2 in AGs with the expression in DNTs. Researchers using samples and data from the Children’s Brain Tumor Network will analyze RNA expression of EAAT2 gene, hoping to better understand its role in epilepsy and possibly identifying new targets for the treatment of seizures and the tumors themselves.

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What are the goals of this project?

Researchers will analyze the role of EAAT2 in the development of seizures in patients with DNTs and AGs.

What is the impact of this project?

This line of research could pave the way for new therapeutic options for children suffering from DNT and AG induced seizures and epilepsy.

Why is the CBTN request important to this project?

AGs are extremely rare tumors, making CBTNs contribution of samples vital to this work.