A genetic mouse model of malignant peripheral nerve sheath tumor with postnatal Nf1 and p53 loss recapitulates the histology and transcriptome of human tumors

We established a new genetically engineered mouse (GEM) model of malignant peripheral nerve sheath tumors (MPNST) based on postnatal deletion of a Nf1;Trp53 cis-conditional allele by the tamoxifen-inducible Plp-CreER (NP-Plp). We also generated two Lats1;2 conditional knockout models by using Nestin-Cre (Lats-Nes) and Plp-CreER (Lats-Plp), both of which also develop tumors similar to MPNST (GEM-PNST). To evaluate these models, transcriptome analyses were performed to compare these models and with human MPNST, plexiform neurofibromas (PNF), and neurofibromas (NF). Transcriptome comparison of various mouse models of maligant peripheral nerve sheath tumor (NP-Plp, NPcis, Lats-Nes, and Lats-Plp) and human MPNST, PNF, and NF tumors.