Chakraborty Ashok, Diwan Anil
AllExcel, Inc. Shelton, CT, USA.
AIMS Neurosci. 2022 Nov 10;9(4):423-443. doi: 10.3934/Neuroscience.2022023. eCollection 2022.
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease in adults involving non-demyelinating motor disorders. About 90% of ALS cases are sporadic, while 10-12% of cases are due to some genetic reasons. Mutations in superoxide dismutase 1 (), , (chromosome 9 open reading frame 72) and genes are commonly found in ALS patients. Therefore, the mechanism of ALS development involves oxidative stress, endoplasmic reticulum stress, glutamate excitotoxicity and aggregation of proteins, neuro-inflammation and defective RNA function. Cholesterol and LDL/HDL levels are also associated with ALS development. As a result, sterols could be a suitable biomarker for this ailment. The main mechanisms of ALS development are reticulum stress, neuroinflammation and RNA metabolism. The multi-nature development of ALS makes it more challenging to pinpoint a treatment.
肌萎缩侧索硬化症(ALS)是一种发生在成年人身上的致命性神经退行性疾病,涉及非脱髓鞘运动障碍。约90%的ALS病例为散发性,而10 - 12%的病例是由某些遗传因素导致的。超氧化物歧化酶1()、(9号染色体开放阅读框72)和基因的突变在ALS患者中较为常见。因此,ALS的发病机制涉及氧化应激、内质网应激、谷氨酸兴奋性毒性和蛋白质聚集、神经炎症以及RNA功能缺陷。胆固醇和低密度脂蛋白/高密度脂蛋白水平也与ALS的发病有关。因此,甾醇可能是这种疾病合适的生物标志物。ALS发病的主要机制是内质网应激、神经炎症和RNA代谢。ALS的多方面发病特性使得确定治疗方法更具挑战性。