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CT-179 + JZP-3507 is an investigational combination therapy being evaluated for the treatment of brain cancers, specifically diffuse midline glioma (DMG). The combination consists of CT-179, a brain-penetrant, orally active small molecule inhibitor of oligodendrocyte transcription factor 2 (OLIG2) developed by Curtana Pharmaceuticals, and JZP-3507 (also known as zeltiprone or ONC206), an orally bioavailable imipridone derivative developed by Jazz Pharmaceuticals that acts as a dual mitochondrial caseinolytic protease P (ClpP) agonist and dopamine receptor D2 (DRD2) antagonist. Preclinical studies in patient-derived models of diffuse midline glioma have demonstrated that combining the OLIG2-targeting mechanism of CT-179 with the mitochondrial stress-inducing and DRD2-antagonizing effects of JZP-3507 results in synergistic anti-tumor efficacy, promoting apoptosis and inhibiting tumor growth.
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