PDF(Pubmed)
Abstract：
Amyotrophic lateral sclerosis (ALS) is a common adult-onset neurodegenerative disease characterized by the progressive death of motor neurons in the cerebral cortex, brain stem, and spinal cord. The exact mechanisms underlying the pathogenesis of ALS remain unclear. The current consensus regarding the pathogenesis of ALS suggests that the interaction between genetic susceptibility and harmful environmental factors is a promising cause of ALS onset. The investigation of putative harmful environmental factors has been the subject of several ongoing studies, but the use of transgenic animal models to study ALS has provided valuable information on the onset of ALS. Here, we review the current common invertebrate genetic models used to study the pathology, pathophysiology, and pathogenesis of ALS. The considerations of the usage, advantages, disadvantages, costs, and availability of each invertebrate model will also be discussed.
摘要：
肌萎缩侧索硬化症（ALS）是一种常见的成人发作的神经退行性疾病，其特征是大脑皮层运动神经元的进行性死亡，脑干,和脊髓。ALS发病机制的确切机制尚不清楚。目前关于ALS发病机制的共识表明，遗传易感性和有害环境因素之间的相互作用是ALS发病的有希望的原因。对假定的有害环境因素的调查一直是几项正在进行的研究的主题，但是使用转基因动物模型研究ALS提供了有关ALS发病的有价值的信息。这里,我们回顾了目前用于研究病理学的常见无脊椎动物遗传模型，病理生理学,和ALS的发病机制。使用的注意事项，优势,缺点,成本,以及每个无脊椎动物模型的可用性也将被讨论。
