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

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.

journal_name

Cancer Cell

journal_title

Cancer cell

authors

Ling BC,Wu J,Miller SJ,Monk KR,Shamekh R,Rizvi TA,Decourten-Myers G,Vogel KS,DeClue JE,Ratner N

doi

10.1016/j.ccr.2004.10.016

keywords:

subject

Has Abstract

pub_date

2005-01-01 00:00:00

pages

65-75

issue

1

eissn

1535-6108

issn

1878-3686

pii

S1535610804003113

journal_volume

7

pub_type

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