NF1^+/- Hematopoietic Cells Accelerate Malignant Peripheral Nerve Sheath Tumor Development without Altering Chemotherapy Response.

ABSTRACT: Haploinsufficiency in the tumor suppressor NF1 contributes to the pathobiology of neurofibromatosis type 1, but a related role has not been established in malignant peripheral nerve sheath tumors (MPNST) where NF1 mutations also occur. Patients with NF1-associated MPNST appear to have worse outcomes than patients with sporadic MPNST, but the mechanism underlying this correlation is not understood. To define the impact of stromal genetics on the biology of this malignancy, we developed unique mouse models that reflect the genetics of patient-associated MPNST. Specifically, we used adenovirus-Cre injections to generate MPNST in Nf1^Flox/Flox; Ink4a/Arf^Flox/Flox and Nf1^Flox/-; Ink4a/Arf^Flox/Flox paired littermate mice to model tumors from NF1-wild-ty

SUBMITTER: Dodd RD 

PROVIDER: S-EPMC5839126 | biostudies-literature | 2017 Aug

REPOSITORIES: biostudies-literature