AIMS Although the pathophysiology of amyotrophic lateral sclerosis (ALS) is still not completely understood, the deregulated microglia polarization and neuroinflammation have been shown to contribute to the pathogenesis and progression of this disease. In the present study, we aimed to determine whether hirsutella sinensis (HS) could reduce neuroinflammatory and pathological changes in the spinal cord of SOD1G93A model mice of ALS and consequently ameliorate disease onset and progression. METHODS SOD1G93A mice were chronically treated with HS by gavage. Their lifespan was recorded, and motor behavior was evaluated by rotarod test. The pathological changes in skeletal muscles and motor neurons in spinal cords were assessed by immunofluorescent staining and hematoxylin-eosin staining. The microglia activation and neuroinflammation were determined by immunofluorescent staining and RT-PCR. RESULTS Our data suggested that repeated HS administration prolonged the lifespan and extended disease duration of ALS mice without significant delay on disease onset. HS ameliorated the pathological changes in the motor neurons and gastrocnemius muscles. Moreover, HS promoted the transition of microglia from pro-inflammatory M1 to anti-inflammatory M2 phenotype in the spinal cord of ALS mice. CONCLUSION All these findings indicate that HS may serve as a potential therapeutic candidate for the treatment of ALS.

中文翻译：

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在SOD1G93A转基因小鼠模型中，hirsutella sinensis对肌萎缩性侧索硬化的发病和进展的治疗作用。

目的尽管肌萎缩性侧索硬化症（ALS）的病理生理学仍未完全了解，但小胶质细胞极化失调和神经炎症已被证明有助于该病的发病和发展。在本研究中，我们旨在确定hirsutella sinensis（HS）是否可以减轻ALS的SOD1G93A模型小鼠脊髓中的神经炎症和病理变化，从而改善疾病的发作和进展。方法采用管饲法对SOD1G93A小鼠进行HS慢性治疗。记录他们的寿命，并通过旋转试验测试运动行为。通过免疫荧光染色和苏木精-曙红染色评估脊髓的骨骼肌和运动神经元的病理变化。通过免疫荧光染色和RT-PCR确定小胶质细胞的活化和神经炎症。结果我们的数据表明，反复给予HS可以延长ALS小鼠的寿命并延长疾病持续时间，而不会显着延迟疾病发作。HS改善了运动神经元和腓肠肌的病理变化。此外，HS促进了ALS小鼠脊髓中小胶质细胞从促炎性M1转变为抗炎性M2的表型。结论所有这些发现表明HS可以作为治疗ALS的潜在候选药物。HS改善了运动神经元和腓肠肌的病理变化。此外，HS促进了ALS小鼠脊髓中小胶质细胞从促炎性M1转变为抗炎性M2的表型。结论所有这些发现表明HS可以作为治疗ALS的潜在候选药物。HS改善了运动神经元和腓肠肌的病理变化。此外，HS促进了ALS小鼠脊髓中小胶质细胞从促炎性M1转变为抗炎性M2的表型。结论所有这些发现表明HS可以作为治疗ALS的潜在候选药物。