Viskochil David H
Department of Pediatrics, School of Medicine, University of Utah, Salt Lake City, Utah 84112, USA.
J Clin Invest. 2003 Dec;112(12):1791-3. doi: 10.1172/JCI20503.
Neurofibromas are benign tumors comprised primarily of Schwann cells and fibroblasts. Mast cell infiltration is a well-known phenomenon; however, their role in tumor pathogenesis has been enigmatic. In an elegant set of experiments using cells derived from a murine model of neurofibromatosis 1 (NF1), Yang et al. dissect the molecular pathways involved in mast cell migration to neurofibromin-deficient Schwann cells. These results set the stage for rational development of therapeutics that could influence the multicellular microenvironment of neurofibromas to inhibit the development and/or progression of these tumors in human NF1.
神经纤维瘤是主要由施万细胞和成纤维细胞组成的良性肿瘤。肥大细胞浸润是一种众所周知的现象;然而,它们在肿瘤发病机制中的作用一直是个谜。在一系列使用源自1型神经纤维瘤病(NF1)小鼠模型的细胞进行的精妙实验中,杨等人剖析了肥大细胞迁移至缺乏神经纤维瘤蛋白的施万细胞所涉及的分子途径。这些结果为合理开发治疗方法奠定了基础,这些治疗方法可能会影响神经纤维瘤的多细胞微环境,从而抑制人类NF1中这些肿瘤的发生和/或进展。