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Tumor microenvironment and neurofibromatosis type I: connecting the GAPs

Oncogene volume 26, pages 4609–4616 (2007)Cite this article

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.

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Acknowledgements

LFP is funded by NINDS and DOD (Grant # DAMD 17-02-1-0638 & DAMD 17-03-1-0216) and ACS RP-04-084-01. We thank members of the Parada lab for helpful discussions and Stephanie Bates for assistance in the preparation of this manuscript.

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Authors and Affiliations

  1. 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

    L Q Le & L F Parada

  2. Department of Dermatology, The University of Texas Southwestern Medical Center, Dallas, TX, USA

    L Q Le

  3. Physician Scientist Training Program, The University of Texas Southwestern Medical Center, Dallas, TX, USA

    L Q Le

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  1. L Q Le
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Correspondence to L Q Le or L F Parada.

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Le, L., Parada, L. Tumor microenvironment and neurofibromatosis type I: connecting the GAPs. Oncogene 26, 4609–4616 (2007). https://doi.org/10.1038/sj.onc.1210261

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