The I, Murthy A E, Hannigan G E, Jacoby L B, Menon A G, Gusella J F, Bernards A
Molecular Neurogenetics Unit, Massachusetts General Hospital, St. Charlestown 02129.
Nat Genet. 1993 Jan;3(1):62-6. doi: 10.1038/ng0193-62.
The introduction of human chromosome 17 suppresses the tumourigenicity of a neuroblastoma cell line in the absence of any effects on in vitro growth and the neurofibromatosis type 1 (NF1) gene may be responsible. Here we report that 4 out of 10 human neuroblastoma lines express little or no neurofibromin and that two of these lines show evidence of NF1 mutations, providing further proof that NF1 mutations occur in tumours that are not commonly found in NF1 patients. We also show that NF1 deficient neuroblastomas show only moderately elevated ras-GTP levels, in contrast to NF1 tumour cells, indicating that neurofibromin contributes differently to the negative regulation of ras in different cell types.
人类17号染色体的导入在不影响体外生长的情况下抑制了神经母细胞瘤细胞系的致瘤性,1型神经纤维瘤病(NF1)基因可能是其原因。我们在此报告,10个人类神经母细胞瘤细胞系中有4个几乎不表达或不表达神经纤维瘤蛋白,其中两个细胞系显示出NF1突变的证据,进一步证明NF1突变发生在NF1患者中不常见的肿瘤中。我们还表明,与NF1肿瘤细胞相比,NF1缺陷的神经母细胞瘤仅显示ras - GTP水平适度升高,这表明神经纤维瘤蛋白在不同细胞类型中对ras的负调控作用不同。