# Journey through tumorverse: Creating models to decode PXA mysteries

**Authors:** George Bukenya, Anthony R. Sloan, Justin D. Lathia

PMC · DOI: 10.1016/j.omton.2024.200853 · Molecular Therapy Oncology · 2024-08-06

## TL;DR

This paper explores the progression of a rare brain tumor in children using a new mouse model to study immune system interactions.

## Contribution

The study introduces a novel immunocompetent mouse model for studying BRAF V600E pediatric low-grade glioma.

## Key findings

- The RCAS-BRAF V600E model recapitulates key features of human PXA tumors.
- The model enables profiling of immunological dynamics in BRAF V600E pLGG.
- It provides insights into tumor progression and immune interactions.

## Abstract

Pleomorphic xanthoastrocytoma (PXA) is a rare pediatric low-grade glioma (pLGG), of which 60%–80% exhibit the BRAF V600E mutation, that enhances the aggressiveness and progression to an eventual pediatric high-grade glioma (pHGG). Despite the aggressiveness of this mutational status, the mechanisms underlying the progression of BRAF V600E tumors remain poorly understood, primarily due to limited insights into their in vivo growth dynamics. In this issue, Rajappa and colleagues leverage a novel immunocompetent RCAS-BRAF V600E murine glioma model to profile the immunological dynamics taking place in BRAF V600E pLGG.

## Linked entities

- **Genes:** BRAF (B-Raf proto-oncogene, serine/threonine kinase) [NCBI Gene 673]
- **Diseases:** pleomorphic xanthoastrocytoma (MONDO:0016690), pediatric high-grade glioma (MONDO:1010030)

## Full-text entities

- **Genes:** Braf (B-Raf proto-oncogene, serine/threonine kinase) [NCBI Gene 109880] {aka 9930012E13Rik, B-raf, Braf-2, Braf2, C230098H17, D6Ertd631e}
- **Diseases:** high-grade glioma (MESH:D008228), glioma (MESH:D005910), tumors (MESH:D009369), PXA (MESH:D001254)
- **Species:** Mus musculus (house mouse, species) [taxon 10090]
- **Mutations:** V600E

## Full text

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## Figures

1 figure with captions in the complete paper: https://tomesphere.com/paper/PMC11347045/full.md

## References

5 references — full list in the complete paper: https://tomesphere.com/paper/PMC11347045/full.md

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Source: https://tomesphere.com/paper/PMC11347045