Role for the epidermal growth factor receptor in neurofibromatosis-related peripheral nerve tumorigenesis

Benjamin C. Ling, Jianqiang Wu, Shyra J. Miller, Kelly R. Monk, Rania Shamekh, Tilat A. Rizvi, Gabrielle Decourten-Myers, Kristine S. Vogel, Jeffrey E. Declue, Nancy Ratner

Research output: Contribution to journalArticle

100 Scopus citations

Abstract

Benign neurofibromas and malignant peripheral nerve sheath tumors are serious complications of neurofibromatosis type 1. The epidermal growth factor receptor is not expressed by normal Schwann cells, yet is overexpressed in subpopulations of Nf1 mutant Schwann cells. We evaluated the role of EGFR in Schwann cell tumorigenesis. Expression of EGFR in transgenic mouse Schwann cells elicited features of neurofibromas: Schwann cell hyperplasia, excess collagen, mast cell accumulation, and progressive dissociation of non-myelin-forming Schwann cells from axons. Mating EGFR transgenic mice to Nf1 hemizygotes did not enhance this phenotype. Genetic reduction of EGFR in Nf1+/-;p53 +/- mice that develop sarcomas significantly improved survival. Thus, gain- and loss-of-function experiments support the relevance of EGFR to peripheral nerve tumor formation.

Original languageEnglish (US)
Pages (from-to)65-75
Number of pages11
JournalCancer Cell
Volume7
Issue number1
DOIs
StatePublished - Jan 1 2005

ASJC Scopus subject areas

  • Oncology
  • Cell Biology
  • Cancer Research

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    Ling, B. C., Wu, J., Miller, S. J., Monk, K. R., Shamekh, R., Rizvi, T. A., Decourten-Myers, G., Vogel, K. S., Declue, J. E., & Ratner, N. (2005). Role for the epidermal growth factor receptor in neurofibromatosis-related peripheral nerve tumorigenesis. Cancer Cell, 7(1), 65-75. https://doi.org/10.1016/j.ccr.2004.10.016