Molecular Mechanisms of Schwann Cell Proliferation in NF1
Abstract
Neurofibromatosis type 1 (NF 1) is a genetic disorder characterized by Schwann cell tumors called neurofibromas, which can potentially become malignant to form neurofibrosarcomas. Previously, our laboratory has reported that Schwann cells derived from neurofibromas abnormally express high levels of the receptor tyrosine kinase c-Kit, which contributes weakly to Schwann cell proliferation (Badache et at, 1998a). It is possible that c-Kit plays an important role during development and that its aberrant re-expression by adult NF 1 cells contributes to the development of Schwann cell tumors. We now report that neurofibromin expression and c-Kit expression are inversely regulated in rat sciatic nerves during development. Normal adult Schwann cells contain neurofibromin and do not express c-Kit, while human Schwann cell lines from NF1 tumors express c-Kit but do not express neurofibromin. In neonates, c-Kit is expressed by cultured Schwann cells. Its activation by SCs prevents programmed cell death via the activation of Akt but does not induce Schwann cell proliferation or differentiation.
Document Details
- Document Type
- Technical Report
- Publication Date
- Sep 01, 2001
- Accession Number
- ADA407274
Entities
People
- George H. Devries
Organizations
- Chicago Association for Research and Education in Science