Amyotrophic lateral sclerosis is a fatal, multigenic, multifactorial neurodegenerative disease characterized by upper and lower motor neuron loss. Animal models are essential for investigating pathogenesis and reflecting clinical manifestations, particularly in developing reasonable prevention and therapeutic methods for human diseases. Over the decades, researchers have established a host of different animal models in order to dissect amyotrophic lateral sclerosis (ALS), such as yeast, worms, flies, zebrafish, mice, rats, pigs, dogs, and more recently, non-human primates. Although these models show different peculiarities, they are all useful and complementary to dissect the pathological mechanisms of motor neuron degeneration in ALS, contributing to the development of new promising therapeutics. In this review, we describe several common animal models in ALS, classified by the naturally occurring and experimentally induced, pointing out their features in modeling, the onset and progression of the pathology, and their specific pathological hallmarks. Moreover, we highlight the pros and cons aimed at helping the researcher select the most appropriate among those common experimental animal models when designing a preclinical ALS study.

中文翻译：

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几种常见肌萎缩侧索硬化症动物模型的用途及优点

肌萎缩侧索硬化症是一种致命的、多基因、多因素的神经退行性疾病，其特征是上、下运动神经元损失。动物模型对于研究发病机制和反映临床表现，特别是对人类疾病制定合理的预防和治疗方法至关重要。几十年来，研究人员建立了许多不同的动物模型来剖析肌萎缩侧索硬化症（ALS），例如酵母、蠕虫、苍蝇、斑马鱼、小鼠、大鼠、猪、狗，以及最近的非人类灵长类动物。尽管这些模型表现出不同的特点，但它们对于剖析 ALS 运动神经元变性的病理机制都是有用且互补的，有助于开发新的有前途的疗法。在这篇综述中，我们描述了几种常见的ALS动物模型，按自然发生的和实验诱导的进行分类，指出它们的建模特点、病理的发生和进展以及具体的病理特征。此外，我们还强调了其优缺点，旨在帮助研究人员在设计临床前 ALS 研究时从常见的实验动物模型中选择最合适的模型。