Oncogenic RABL6A promotes NF1-associated MPNST progression in vivo.

ABSTRACT:

Background

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive sarcomas with complex molecular and genetic alterations. Powerful tumor suppressors CDKN2A and TP53 are commonly disrupted along with NF1, a gene that encodes a negative regulator of Ras. Many additional factors have been implicated in MPNST pathogenesis. A greater understanding of critical drivers of MPNSTs is needed to guide more informed targeted therapies for patients. RABL6A is a newly identified driver of MPNST cell survival and proliferation whose in vivo role in the disease is unknown.

Methods

Using CRISPR-Cas9 targeting of Nf1 + Cdkn2a or Nf1 + Tp53 in the mouse sciatic nerve to form de novo MPNSTs, we investigated the biological significance

SUBMITTER: Kohlmeyer JL 

PROVIDER: S-EPMC9092646 | biostudies-literature | 2022 Jan-Dec

REPOSITORIES: biostudies-literature