Funded by the Dick Vitale Pediatric Cancer Research Fund
Diffuse midline glioma (DMG) is a serious brain tumor that mainly affects children. These tumors often carry a genetic change called H3K27M, which helps drive the disease. However, we still do not fully understand how DMG cells use nutrients to support their growth.Our early research shows that DMG cells use unusually high amounts of three amino acids—leucine, isoleucine, and valine. These nutrients are known as branched-chain amino acids, or BCAAs. We also found high levels of BCAT1, an enzyme that helps break down BCAAs. When BCAT1 is reduced, DMG cells do not grow as well in the laboratory.This study will examine why DMG tumors depend on BCAAs and BCAT1. We will track how tumor cells use these nutrients and study what happens when BCAT1 is blocked. We will also test whether lowering BCAAs in the diet could slow tumor growth. Similar diets are already used safely in children with certain metabolic disorders.Our goal is to identify a new way to limit DMG growth and, ultimately, help patients live longer.