Tumor microenvironment and neurofibromatosis type I: Connecting the GAPs

L. Q. Le, L. F. Parada

Research output: Contribution to journalReview article

126 Scopus citations

Abstract

The human disease von Recklinghausen's neurofibromatosis (Nf1) is one of the most common genetic disorders. It is caused by mutations in the NF1 tumor suppressor gene, which encodes a GTPase activating protein (GAP) that negatively regulates p21-RAS signaling. Dermal and plexiform neurofibromas as well as malignant peripheral nerve sheath tumors and other malignant tumors, are significant complications in Nf1. Neurofibromas are complex tumors and composed mainly of abnormal local cells including Schwann cells, endothelial cells, fibroblasts and additionally a large number of infiltrating inflammatory mast cells. Recent work has indicated a role for the microenvironment in plexiform neurofibroma genesis. The emerging evidence points to mast cells as crucial contributors to neurofibroma tumorigenesis. Therefore, further understanding of the molecular interactions between Schwann cells and their environment will provide tools to develop new therapies aimed at delaying or preventing tumor formation in Nf1 patients.

Original languageEnglish (US)
Pages (from-to)4609-4616
Number of pages8
JournalOncogene
Volume26
Issue number32
DOIs
StatePublished - Jul 12 2007

Keywords

  • GAP
  • Mast cell
  • NF1
  • Neurofibroma
  • Neurofibromin
  • Tumor microenvironment

ASJC Scopus subject areas

  • Molecular Biology
  • Genetics
  • Cancer Research

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