Rao Mala V, Nixon Ralph A
Center for Dementia Research, Nathan Kline Institute/Department of Psychiatry, NYU School of Medicine, 140 Old Orangeburg Road, Orangeburg, New York 10962, USA.
Neurochem Res. 2003 Jul;28(7):1041-7. doi: 10.1023/a:1023259207015.
Neurofilament proteins synthesized in the cell body of neurons are assembled and transported into axons, where they influence axon radial growth, axonal transport, and nerve conduction velocities. In diseased states, neurofilaments accumulate in cell bodies and proximal axons of affected neurons, and these lesions are characteristic of many neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), Alzheimer's disease (AD), spinal muscular atrophy (SMA), Charcot-Marie-Tooth disease type 2 (CMT2), and hereditary sensory motor neuropathy. Although the molecular mechanisms that contribute to these accumulations are not yet identified, transgenic mouse models are beginning to provide insight into the role of neurofilament transport in disease-related dysfunction of neurons. This review addresses axonal transport in mouse models of ALS and the special significance of neurofilament transport in this disease.
在神经元胞体中合成的神经丝蛋白会进行组装并运输到轴突中，在轴突中它们会影响轴突的径向生长、轴突运输以及神经传导速度。在患病状态下，神经丝会在受影响神经元的胞体和近端轴突中积累，这些病变是许多神经退行性疾病的特征，包括肌萎缩侧索硬化症（ALS）、阿尔茨海默病（AD）、脊髓性肌萎缩症（SMA）、2型夏科-马里-图斯病（CMT2）以及遗传性感觉运动神经病。尽管导致这些积累的分子机制尚未明确，但转基因小鼠模型开始为深入了解神经丝运输在神经元疾病相关功能障碍中的作用提供线索。本综述探讨了ALS小鼠模型中的轴突运输以及神经丝运输在该疾病中的特殊意义。
