神经纤维瘤病
癌变
神经纤维瘤病
神经系统
生物
表型
2型神经纤维瘤病
雪旺细胞
外周神经系统
神经科学
病理
癌症
中枢神经系统
医学
遗传学
基因
解剖
出处
期刊:Annual Review of Pathology-mechanisms of Disease
[Annual Reviews]
日期:2007-01-23
卷期号:2 (1): 191-216
被引量:128
标识
DOI:10.1146/annurev.pathol.2.010506.091940
摘要
As familial cancer syndromes, the neurofibromatoses exhibit complex phenotypes, comprising a range of tumor and nontumor manifestations. Although the three recognized forms of neurofibromatosis (NF1, NF2, and schwannomatosis) all feature the development of nervous system tumors, their underlying genetic bases are clearly distinct. The most prominent common feature of all three is the appearance of Schwann cell–initiated tumorigenesis of the peripheral nervous system. Recent progress in delineating the molecular function of the NF1- and NF2-encoded proteins, together with the development and use of manipulable mouse models, has led to important advances in understanding the pathogenesis of many features of neurofibromatosis. An important outcome of the study of neurofibromatosis-associated tumorigenesis has been insight into the more general molecular and cellular bases of nervous system tumors.
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