Journal
ONCOGENE
Volume 26, Issue 32, Pages 4609-4616Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/sj.onc.1210261
Keywords
neurofibromin; tumor microenvironment; mast cell; neurofibroma; NF1; GAP
Funding
- NINDS NIH HHS [P50 NS052606, P50 NS052606-05] Funding Source: Medline
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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.
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