肌萎缩侧索硬化
SOD1
超氧化物歧化酶
兴奋毒性
病理
医学
利鲁唑
萎缩
皮质脊髓束
神经科学
氧化应激
生物
谷氨酸受体
疾病
内科学
磁共振成像
受体
磁共振弥散成像
放射科
作者
Xinxin Wang,Wenzhi Chen,Cheng Li,Renshi Xu
出处
期刊:Reviews in The Neurosciences
[De Gruyter]
日期:2024-02-22
标识
DOI:10.1515/revneuro-2024-0010
摘要
Abstract Amyotrophic lateral sclerosis (ALS) is a rare neurodegenerative disease which damages upper and lower motor neurons (UMN and LMN) innervating the muscles of the trunk, extremities, head, neck and face in cerebrum, brain stem and spinal cord, which results in the progressive weakness, atrophy and fasciculation of muscle innervated by the related UMN and LMN, accompanying with the pathological signs leaded by the cortical spinal lateral tract lesion. The pathogenesis about ALS is not fully understood, and no specific drugs are available to cure and prevent the progression of this disease at present. In this review, we reviewed the structure and associated functions of copper-zinc superoxide dismutase 1 (SOD1), discuss why SOD1 is crucial to the pathogenesis of ALS, and outline the pathogenic mechanisms of SOD1 in ALS that have been identified at recent years, including glutamate-related excitotoxicity, mitochondrial dysfunction, endoplasmic reticulum stress, oxidative stress, axonal transport disruption, prion-like propagation, and the non-cytologic toxicity of glial cells. This review will help us to deeply understand the current progression in this field of SOD1 pathogenic mechanisms in ALS.
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