肌萎缩性侧索硬化症(ALS)的硫化氢“双面两面”

P. Longone, A. Davoli, V. Greco, A. Spalloni, E. Guatteo, C. Nerí, Giada Ricciardo Rizzo, Alberto Cordella, A. Romigi, C. Cortese, S. Bernardini, P. Sarchielli, G. Cardaioli, P. Calabresi, A. Urbani, N. Mercuri
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引用次数: 3

摘要

我们最近在《神经学年鉴》上发表了一篇论文,题为《硫化氢参与肌萎缩性侧索硬化症的证据》,其中我们报告了一项对患者和家族性ALS遗传模型的研究。这项研究的结果是一个原始的发现:在人类患者和动物模型中过量产生硫化氢(h2s)。我们还发现,h2s主要由神经胶质细胞产生,对运动神经元有毒性,并显著增加胞浆ca2 +浓度。总之,我们的数据介绍了h2s作为ALS相关毒性途径的新竞争者,这对ALS的创新药物设计具有潜在的意义。
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Hydrogen sulphide "a double-faced Janus" in amyotrophic lateral sclerosis (ALS)
We have recently published a paper in Annals of Neurology entitled “Evidence of Hydrogen Sulphide involvement in Amyotrophic Lateral Sclerosis” [1] where we reported a study performed in patients, and in a genetic model of familial ALS. The outcome of this study is an original finding: the overproduction of hydrogen sulphide (H 2 S) in the human patients and in the animal model. We also show that H 2 S is produced, mainly, by glial cells, is toxic to motor neurons and increases significantly cytosolic Ca 2+ concentration. Altogether, our data introduce H 2 S as a new contestant in the ALS-related toxic pathways, which has potential implications for innovative drug design in ALS.
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