Tumor microenvironment and neurofibromatosis type I: connecting the GAPs

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