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用铜化合物CuATSM治疗对ALS患者的运动神经元病理没有显著影响

Yue Yang^1,2, Dominic Rowe³, Heather McCann⁴

¹Brain and Mind Centre, University of Sydney, Sydney, New South Wales, Australia.

Neuropathology and applied neurobiology

|June 15, 2023

概括

铜化合物CuATSM在小鼠模型中表现有希望,但没有显著改善神经元密度或TDP-43负担在肌缩性侧面硬化症 (ALS) 患者. 这项研究表明,CuATSM在人类ALS病例中并不能缓解神经元病理.

科学领域:

神经科学是一个神经科学.
神经学神经学
病理学病理学病理学

背景情况:

肌缩侧面硬化症 (ALS) 是一种进展性神经退行性疾病.
像CuATSM这样的铜化合物在临床前的ALS模型中显示出治疗潜力.
在人类ALS患者中CuATSM的疗效尚不清楚.

研究的目的:

调查CuATSM对人类患者ALS病理学的影响.
为了比较用CuATSM和riluzole治疗的ALS患者的神经病理变化与单独使用riluzole.

主要方法:

试点对死后大脑和脊髓组织进行比较分析.
分析包括患有ALS-TDP和ALS-SOD的患者1.
用CuATSM和riluzole治疗的病例与仅用riluzole治疗的病例进行比较.

主要成果:

两组之间没有观察到运动皮层或脊髓神经元密度或TDP-43负担的显著差异.
CuATSM治疗与运动皮质中的p62-免疫反应性星球细胞有关,并减少了脊髓中的Iba1密度.
在CuATSM的使用中,没有发现天体细胞活性或SOD1免疫反活性的显著变化.

结论:

在人类ALS患者中,CuATSM并没有显著缓解神经元病理或星病.
这些发现与临床前ALS模型的有希望的结果形成鲜明对比.
这项研究提供了第一个死后证据,证明CuATSM在人类ALS中有效性有限.

关键词:

在TDP-43病理学方面,骨髓缩侧面硬化症 (ALS) 是一种铜ATSM (CuATSM) 的使用 p62 病理学病理学瑞卢 (riluzole) 是一种药物.

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