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PMID: 17297459 Published · ppublish English Journal Article Research Support, N.I.H., Extramural Research Support, Non-U.S. Gov't Research Support, U.S. Gov't, Non-P.H.S. Review

Tumor microenvironment and neurofibromatosis type I: connecting the GAPs.

Oncogene ·Vol. 26 ·No. 32 ·2007-07-12 ·Pages 4609-16

Le LQ, Parada LF

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.

MeSH Terms
Animals Genes, Neurofibromatosis 1 Humans Mast Cells/enzymology,physiology Mice Neurofibromatosis 1/etiology,genetics,therapy Neurofibromin 1/genetics,physiology
Chemicals
Neurofibromin 1
Authors & Affiliations
2 authors, click to expand affiliations / ORCID
Le L Q
Department of Developmental Biology and Kent Waldrep Foundation Center for Basic Research on Nerve Growth and Regeneration, The University of Texas Southwestern Medical Center, Dallas, TX, USA. Lu.Le@UTSouthwestern.edu
Parada L F
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Article Info
Journal
Oncogene
Abbr.
Oncogene
ISSN
0950-9232
DOI
10.1038/sj.onc.1210261
Email
lu.le@utsouthwestern.edu
Published
2007-07-12
Epub
2007-00-12
Pages
4609-16
Language
English
Region
England
NLM ID
8711562
PMCID
PMC2760340
Subset
IM
Grants
NINDS NIH HHS · P50 NS052606 · United States
NINDS NIH HHS · P50 NS052606-05 · United States
External Links
PubMed source DOI: 10.1038/sj.onc.1210261 PMC Full Text View journal details
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