Notch Signaling and Schwann Cell Transformation: Development of a Model System and Application to Human MPNSTs

摘要

This is a final report that presents data obtained during the grant's duration of funding. The grant addresses the potential role of Notch signaling in the malignant transformation of neurofibromas to MPNSTs in patients with NF1. Our previous work has shown that constitutive expression of Notch can transform rat Schwann cells and that at least on MPNST-derived human Schwann cell line (of three examined) signals via Notch. This report includes results pertaining to the Tasks of the Statement of Work, including our observation that NICD is NOT sufficient to transform primary Schwann cells. This led to a new Task that addressed this discrepancy and results that showed that NICD provides a Raslike activity for the transformation of Schwann cells.