Glucose transport across the blood-brain barrier and into astrocytes relies on the glucose transporter type 1 (GLUT1). Heterozygous mutations in SLC2A1 cause GLUT1 Deficiency Syndrome (GLUT1-DS), a rare disorder that affects brain energy metabolism and development. Despite the genetic cause being identified over three decades ago, no therapies beyond the ketogenic diet exist. A US-based biotechnology company has been developing a small molecule that boosts astrocytic mitochondrial ATP production; its name is withheld due to an ongoing patent application. We aim to evaluate this compound as a candidate therapy for GLUT1-DS. To mimic GLUT1-DS, primary human astrocytes were incubated in glucose-free medium and treated with the compound, β-hydroxybutyrate (βHB), or their combination. These treatments significantly increased intracellular ATP individually and had a synergistic effect when combined. Mitochondrial membrane potential was also enhanced with either treatment. Additionally, intracellular calcium imaging revealed that the compound further increased Ca²⁺ fluctuations in reactive astrocytes versus controls, suggesting enhanced responsiveness under pathological conditions. These results support the potential of this compound as a pharmacological therapy to enhance astrocyte metabolism in GLUT1-DS. Next, we will test it in hiPSC-derived astrocytes modeling SLC2A1 haploinsufficiency and in transgenic mice to generate translational preclinical evidence.