Motor neuron disease and model systems: aetiologies, mechanisms and therapies.
Motor neuron disease and model systems: aetiologies, mechanisms and therapies.
复制标题
运动神经元疾病和模型系统：病因、机制和治疗。
DOI：
10.1002/9780470514863.ch2
复制
发表时间：
1996
期刊：
影响因子：
--
通讯作者：
Sisodia,SS
中科院分区：
文献类型：
--
作者：
Price,DL;Koliatsos,VE;Wong,PC;Pardo,CA;Borchelt,DR;Lee,MK;Cleveland,DW;Griffin,JW;Hoffman,PN;Cork,LC;Sisodia,SS
许多神经系统疾病的表型，包括运动神经元疾病（肌萎缩侧索硬化症）和阿尔茨海默病（AD），是由神经细胞群体的脆弱性和细胞异常的特征/演变决定的。由于不同的细胞类型对单个营养因子有选择性的反应，这些因子可能有助于改善表达其同源受体的细胞的病理。为了测试ALS和AD的治疗方法，研究人员需要模型系统。尽管ALS的模型多种多样，但有两种模型特别有吸引力：表达与家族性ALS相关的人超氧化物歧化酶1 （SOD‐1）突变的转基因小鼠出现与突变SOD的不利特性相关的瘫痪；以及新生大鼠面部轴突的轴突切开术，这是一种引起逆行细胞变性的操作，可以通过几种营养因子改善。
The phenotypes of many neurological diseases, including motor neuron disease (amyotrophic lateral sclerosis; ALS) and Alzheimer's disease (AD), are determined by the vulnerabilities of populations of nerve cells and the character/evolution of cellular abnormalities. Because different cell types respond selectively to individual trophic factors, these factors may be useful in ameliorating pathology in cells that express their cognate receptors. To test therapies for ALS and AD, investigators require model systems. Although there are a variety of models of ALS, two models are particularly attractive: transgenic mice that express human superoxide dismutase 1 (SOD‐1) mutations linked to familial ALS develop paralysis associated with a gain of adverse property of the mutant SOD; and axotomy of facial axons in neonatal rats, a manipulation that causes retrograde cell degeneration, which can be ameliorated by several trophic factors.