Current Therapy of Drugs in Amyotrophic Lateral Sclerosis

被引:38
作者
Lu, Haiyan [1 ]
Le, Wei Dong [2 ]
Xie, Ya-Ying [1 ]
Wang, Xiao-Ping [1 ]
机构
[1] Shanghai Jiao Tong Univ, Dept Neurol, Shanghai Peoples Hosp 1, Shanghai 200080, Peoples R China
[2] Baylor Coll Med, Dept Neurol, Houston, TX 77030 USA
基金
中国国家自然科学基金;
关键词
Amyotrophic lateral sclerosis; motor neuron disease; neurodegenerative disease; SOD1; mutations; riluzole; edaravone; pyrimethamine; TRANSGENIC MOUSE MODEL; DELAYS DISEASE ONSET; SUPEROXIDE-DISMUTASE; CEREBROSPINAL-FLUID; EXTENDS SURVIVAL; VALPROIC ACID; MUTANT SOD1; NEUROPROTECTIVE AGENTS; PROTEIN AGGREGATION; MOTOR-NEURONS;
D O I
10.2174/1570159X14666160120152423
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
Amyotrophic lateral sclerosis (ALS), commonly termed as motor neuron disease (MND) in UK, is a chronically lethal disorder among the neurodegenerative diseases, meanwhile. ALS is basically irreversible and progressive deterioration of upper and lower motor neurons in the motor cortex, brain stem and medulla spinalis. Riluzole, used for the treatment of ALS, was demonstrated to slightly delay the initiation of respiratory dysfunction and extend the median survival of patients by a few months. In this study, the key biochemical defects were discussed, such as: mutant Cu/Zn superoxide dismutase, mitochondrial protectants, and anti-excitotoxic/ anti-oxidative / anti-inflammatory/ anti-apoptotic agents, so the related drug candidates that have been studied in ALS models would possibly be further used in ALS patients.
引用
收藏
页码:314 / 321
页数:8
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