A therapeutic window for preventive therapy in NF1-associated optic pathway glioma

Yuan Zhu; Wang Zheng; Emmanuelle S Jecrois; Brianna R Pierce; Daniel M Treisman

doi:10.1080/23723556.2021.1989262

A therapeutic window for preventive therapy in NF1-associated optic pathway glioma

Mol Cell Oncol. 2021 Nov 30;8(6):1989262. doi: 10.1080/23723556.2021.1989262. eCollection 2021.

Authors

Yuan Zhu^{1

2

3

4}, Wang Zheng^{1

2

3}, Emmanuelle S Jecrois^{1

2

3

4}, Brianna R Pierce^{1

2

3}, Daniel M Treisman^{1

2

3}

Affiliations

¹ Gilbert Family Neurofibromatosis Institute, Children's National Hospital, Washington, DC, USA.
² Center for Cancer and Immunology Research, Children's National Hospital, Washington, DC, USA.
³ Center for Neuroscience Research, Children's National Hospital, Washington, DC, USA.
⁴ Neuroscience Graduate Program, University of Michigan Medical School, Ann Arbor, MI, USA.

Abstract

Pediatric low-grade gliomas (pLGGs) are almost universally driven by abnormal activation of RAS-mediated MEK-ERK/MAPK signaling pathway. pLGGs predominantly occur in children, suggesting that they originate in an ERK-dependent neural stem/progenitor population(s) transiently present in the developing brain. Our recent preclinical study reveals a cell-lineage-of-origin and develops a chemopreventative therapeutic strategy.

Keywords: Neurofibromatosis type 1; hypothalamic ventricular zone (hVZ); neural stem cells; pediatric low-grade glioma; radial glia (RG).

Grants and funding

This work was supported by the DOD NFRP (W81XWH-17-1-0083) and the Gilbert Family Neurofibromatosis Institute.