College of Pharmacy and Research Center for Cell Fate Control, Sookmyung Women's University, Seoul, South Korea.
Adv Exp Med Biol. 2017;975 Pt 2:887-895. doi: 10.1007/978-94-024-1079-2_70.
Amyotrophic lateral sclerosis (ALS) is a fetal neurodegenerative disease that results in motor dysfunction and death. However, there is no cure or effective therapy for ALS. In our previous results, taurine protects motor neurons by repairing for constitutive oxidative stress in an ALS model. ALS is caused by multiple factors including inflammation, oxidative stress, mitochondrial dysfunction, apoptosis, glutamate excitotoxicity and proteasomal dysfunction. Especially, glutamate excitotoxicity has been well known as a mediator in the disease process, and may occur from changes in the excitability of the neurons being stimulated. D-serine is known to a key factor of determination on glutamate toxicity in ALS. Therefore, in the present study, we investigated neuroprotective effects of taurine from glutamate excitotoxicity using motor neuron cells, mtSOD1 (G93A) transgenic cell line model of ALS (NSC-34/hSOD1G93A cells). We evidenced that taurine protects cultured motor neurons from neurotoxic injury. Our findings indicated that taurine has neuroprotective properties and may be a good candidate for therapeutic trials in ALS.
肌萎缩侧索硬化症（ALS）是一种胎儿神经退行性疾病，可导致运动功能障碍和死亡。然而，目前尚无针对 ALS 的治愈方法或有效疗法。在我们之前的研究结果中，牛磺酸通过修复 ALS 模型中的组成性氧化应激来保护运动神经元。ALS 是由多种因素引起的，包括炎症、氧化应激、线粒体功能障碍、细胞凋亡、谷氨酸兴奋性毒性和蛋白酶体功能障碍。特别是，谷氨酸兴奋性毒性已被认为是疾病过程中的一种介导物，可能是由于被刺激的神经元的兴奋性发生变化而产生的。D-丝氨酸被认为是 ALS 中谷氨酸毒性的决定因素之一。因此，在本研究中，我们使用运动神经元细胞、mtSOD1（G93A）转基因细胞系 ALS 模型（NSC-34/hSOD1G93A 细胞）研究了牛磺酸对谷氨酸兴奋性毒性的神经保护作用。我们证明了牛磺酸可保护培养的运动神经元免受神经毒性损伤。我们的研究结果表明，牛磺酸具有神经保护特性，可能是 ALS 治疗试验的良好候选药物。
