The Study On The Construction Of Neurofibromas Cell Model In NF1 With RNAi Technology

1. BackgroundsNeurofibromatosis type 1(NF1) is an autosomal dominant disorder,characterized by multiple systematic damage derived from the abnormal differentiation of the neural crest cells.The symptom of multiple neurofibromas is one of the most characteristic clinical features of NF1,which are benign heterogenic tumors mainly consisting of Schwann cells and fibroblasts. Recent researching reports prone to support the hypothesis that they come from Schwann cells and the previous experiments of our researching group also testified it.The NF1 gene is a big gene ,its product, neurofibromin, contains a region homologous to yeast IRA proteins and mammalian Ras-GTPase-activating proteins that function as negative regulators of Ras by accelerating the conversion of Ras-GTP to Ras-GDP. Lowering Ras-GTP in these cells inhibits cell growth. According to the previous researches on NF1,we know that the increased levelof RasGTP in Schwann cells resulting from the mutation of NF1 gene play a major role in the pathogenesis of neurofibromas in patients with NF1,but its definite pathogenesis is still unknown..RNA interference (RNAi) is the phenomenon of sequence-specific post-transcriptional gene silencing in a wide range of organisms developed in recent years, initiated by double-stranded RNA (dsRNA) that is homologous in sequence to the targeting gene. Meanwhile,it also is a significant method of inhibiting sequence-specific gene expression.Researchers began using this technology to analyze...