Institute of Neurology, Ruijin Hospital, Shanghai JiaoTong University School of Medicine, Shanghai, 200025, PR China.
Engineering Research Center of Modern Preparation Technology of TCM, Shanghai University of Traditional Chinese Medicine, Shanghai, 201203, PR China.
Sci Rep. 2018 Jan 26;8(1):1668. doi: 10.1038/s41598-018-19923-9.
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease characterized by progressive loss of motor neurons. There are no definitive pathogenic mechanisms and effective treatments for ALS now. Traditional Chinese medicine (TCM) plays an important role in Chinese health care system. Huolingshengji Formula (HLSJ) is a TCM formula which is applied for treating flaccid syndrome. Our previous clinical study has indicated that HLSJ may have therapeutic effects in ALS patients. In the present study, we analyzed the chemical profile of HLSJ by the high-performance liquid chromatographic (HPLC) fingerprint analysis. And we investigated the therapeutic effects and neuroprotective mechanisms of HLSJ against ALS in SOD1 mouse model. Eleven typical peaks were identified by the fingerprint analysis of HLSJ, and the HPLC method had good precision, repeatability and stability. Consistent with our clinical studies, HLSJ significantly prolonged the lifespan, extended the disease duration, and prevented the motor neuron loss in the anterior horn of the lumbar spinal cords in SOD1 ALS model mice. Additionally, HLSJ alleviated the atrophy of the gastrocnemius muscles and ameliorated the apoptotic and inflammatory levels in the spinal cords of SOD1 mice. Collectively, our study indicated that HLSJ might be a novel candidate for the treatment of ALS.
肌萎缩侧索硬化症（ALS）是一种致命的神经退行性疾病，其特征是运动神经元进行性丧失。目前，ALS 的发病机制尚未明确，也没有有效的治疗方法。中医药在我国的医疗保健体系中发挥着重要作用。虎羚圣吉方（HLSJ）是一种用于治疗痿证的中药方剂。我们之前的临床研究表明，HLSJ 可能对 ALS 患者具有治疗作用。在本研究中，我们采用高效液相色谱（HPLC）指纹分析方法分析了 HLSJ 的化学成分。并在 SOD1 小鼠模型中研究了 HLSJ 治疗 ALS 的疗效和神经保护机制。通过 HLSJ 的指纹分析鉴定出 11 个典型峰，HPLC 方法具有良好的精密度、重复性和稳定性。与我们的临床研究一致，HLSJ 显著延长了 SOD1 ALS 模型小鼠的寿命，延长了疾病持续时间，并防止了腰椎脊髓前角运动神经元的丢失。此外，HLSJ 减轻了 SOD1 小鼠腓肠肌的萎缩，并改善了脊髓中的细胞凋亡和炎症水平。综上所述，我们的研究表明 HLSJ 可能是治疗 ALS 的一种新型候选药物。
