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A novel model of pediatric glioma of H3F3A mutant and TP53 mutant cerebral organoids

The data associated with this publication are not available for this reason: N/A
Abstract

Diffuse Intrinsic Pontine Glioma (DIPG) is an incurable childhood brainstem tumor, affecting 200-400 children in the United States per year. Once diagnosed, the only known treatment is radiation, with death occurring in ​<12 months of initial diagnosis.

Resection is impossible due to its location and infiltrative growth. Chemotherapeutic agents are ineffective due to poor understanding of underlying molecular and cellular biology, and lack of in-vitro and in-vivo models for testing.

In patients with DIPG, genetic mutations of gain-of-function K27M of Histone H3.3 gene (H3F3A) and loss-of-function P53 gene (TP53) frequently co-occur.

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