Ⅰ型神经纤维瘤病神经纤维形成机制的初步研究

目的研究I型神经纤维瘤病(NF1)神经纤维瘤与非NF1神经纤维瘤的发病机制的异同,证实雪旺 细胞在NF1神经纤维瘤发病中的重要作用。方法用S100与NF1蛋白的特异性抗体,通过免疫组织化学分别检 测13例NF1患者与10例非NF1患者的神经纤维瘤中S100,NF1蛋白的表达,分析NF1与非NF1组神经纤维瘤中 雪旺细胞,纤维母细胞的含量及NF1蛋白表达缺失的比率。结果NF1组神经纤维中雪旺细胞与纤维母细胞的平 均含量为66．41％,29．4％,两者差异有显著性意义(t=6．024,P<0．05);雪旺细胞与纤维母细胞的NF1蛋白表达 缺失率分别为64．18％,50．16％,两者差异有显著性意义(t=3．225,P<0．01);而NF1蛋白表达缺失的雪旺细胞与 纤维母细胞的平均含量分别为43．3％,14．9％,两者差异也有显著性意义(t=4．358,P<0．01),说明雪旺细胞为 NF1患者神经纤维瘤形成的主要细胞,非NF1组神经纤维瘤中雪旺细胞与纤维母细胞均未检测到NF1蛋白的表达 缺失,与NF1组相比,两者的NF1蛋白表达率差异有显著性意义(t=10．05,P<0．01),说明两组神经纤维瘤的发病 机制是完全不同的。结论NF1与非NF1神经纤维瘤发病机制完全不同,雪旺细胞的NF1蛋白表达缺失是NF1神 经纤维瘤形成的主要机制。

The preliminary study on the pathogenesis of neurofibromas in neurofibromatosis type I

Objective To find out the differences between neurofibromas of NFl and non-NFl and testify the important function of schwann cells in pathogenesis of neurofibromas in NF1. Methods The expressions of S100 proteins and neurofibromin were investigated in neurofibromas of 13 NF1 patients and 10 non-NF1 patients by immunohistochemistry with antibodies of S100 protein and neurofibromin. The ratios of loss of neurofibromin were analyzed in schwann cells and fibro-blasts. Results The average ratio of schwann cells and fibrobolasts in neurofibromas of NFl were separately 66. 41% and 29. 4% , the difference between them was remarkably significant( t=6. 024, P < 0. 05 ) . The average ratio of loss of neurofibromin in schwann cells and fibroblasts in NFl group were 64. 18% and 50. 16% separately, the difference between them was remarkably significant (t =3. 225,P <0. 01). The average ratios of NFl schwann cells and NFl fibroblasts expression of neurofibromin were 43. 3% and 14. 9%,the difference was remarkably significant (t =4. 358 ,P <0. 01 ). Thus schwann cells are the important and primary cells to form neurofibromas of NF1. No any loss of neurofibromin in schwann cells and fibroblasts in non - NF1 groups was detected, their expression has remarkable significance (t = 10. 05 ,P <0. 01). Conclusions The pathogenesis of neurofibromas in NF1 and non-NF1 is completely different. The loss of neurofibromin in schwann cells is the primary mechanism to form neurofibromas in NF1.