Institute Pasteur Cenci Bolognetti, Istituto Italiano di Tecnologia, Department of Anatomy, Histology, Forensic Medicine and Orthopedics - Unit of Histology and Medical Embryology, Istituto Interuniversitario di Miologia, Sapienza University of Rome, Italy.
FEBS J. 2013 Sep;280(17):4315-22. doi: 10.1111/febs.12087. Epub 2013 Jan 3.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease associated with motor neuron degeneration, muscle atrophy and paralysis. Although numerous pathological mechanisms have been elucidated, ALS remains an invariably fatal disease in the absence of any effective therapy. The heterogeneity of the disease and the failure to develop satisfactory therapeutic protocols reinforce the view that ALS is a multi-factorial and multi-systemic disease. Thus, a better understanding of the pathogenic mechanisms and study of the potential pathological relationship between the various cellular processes is required to ensure efficacious therapy. The pathogenic mechanisms associated with ALS are reviewed, and the strengths and limitations of some new therapeutic approaches are discussed.
肌萎缩侧索硬化症(ALS)是一种致命的神经退行性疾病,与运动神经元变性、肌肉萎缩和瘫痪有关。尽管已经阐明了许多病理机制,但在没有任何有效治疗方法的情况下,ALS 仍然是一种致命的疾病。疾病的异质性和未能制定出令人满意的治疗方案,这进一步表明 ALS 是一种多因素和多系统疾病。因此,需要更好地了解发病机制,并研究各种细胞过程之间潜在的病理关系,以确保有效的治疗。本文综述了与 ALS 相关的发病机制,并讨论了一些新的治疗方法的优缺点。