Lynch Timothy M, Gutmann David H
Department of Neurology, Washington University School of Medicine, Neurofibromatosis Program, St. Louis Children's Hospital, St. Louis, MO, USA.
Neurol Clin. 2002 Aug;20(3):841-65. doi: 10.1016/s0733-8619(01)00019-6.
Neurofibromatosis 1 is one of the most common genetic conditions affecting the nervous system. Individuals with NF1 are predisposed to the development of peripheral nerve sheath tumors (neurofibromas and MPNSTs), astrocytomas (optic pathway gliomas), learning disabilities, seizures, strokes, macrocephaly, and vascular abnormalities. The NF1 tumor suppressor gene encodes a large protein (neurofibromin) that functions primarily as a RAS negative regulator, suggesting that targeted therapy for NF1 might derive from inhibition of the RAS signaling pathway.
神经纤维瘤病1型是影响神经系统的最常见遗传疾病之一。患有神经纤维瘤病1型的个体易患周围神经鞘瘤(神经纤维瘤和恶性外周神经鞘膜瘤)、星形细胞瘤(视路胶质瘤)、学习障碍、癫痫、中风、巨头畸形和血管异常。神经纤维瘤病1型肿瘤抑制基因编码一种大型蛋白质(神经纤维瘤蛋白),其主要功能是作为RAS负调节因子,这表明针对神经纤维瘤病1型的靶向治疗可能源于对RAS信号通路的抑制。