Huntington's disease (HD) is inherited neurodegenerative disease, it is characterized by excessive motor movements and cognitive and emotional deficits. HD is caused by an abnormally long polyglutamine (polyQ) expansion in the huntingtin (Htt) protein, which confers toxic functions to mutant Htt leading to neurodegeneration. Rutin is a flavonoid found in plants, buckwheat, some teas and also in apples. Although previous studies have already indicated that rutin has some protective effects in HD's models, the underlying mechanisms are still unknown. In our study, we investigated the effects of rutin in Caenorhabditis elegans model of HD. We assessed polyQ aggregation, oxidative damage, neurodegeneration level and lifespan, and investigated the possible role of autophagy and insulin/IGF1 (IIS) signaling pathways in the beneficial effects induced by rutin. Overall, our data demonstrate that chronic rutin treatment reduced polyglutamine (polyQ) protein aggregation in muscle, reduced polyQ-mediated neuronal death in ASH sensory neurons, and extended lifespan. The possible mechanisms involved are antioxidant activity, activation of protein degradation (autophagy) and insulin/IGF1 (IIS) signaling pathways. These findings indicate that rutin consumption might be helpful in preventing HD and also provide possible pathways to be explored to search for new therapies against proteinopathies related to aging.

中文翻译：

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芦丁通过胰岛素/ IGF1（IIS）信号传导途径和自噬活性来保护亨廷顿舞蹈病：秀丽隐杆线虫模型的研究。

亨廷顿舞蹈病（HD）是遗传性神经退行性疾病，其特征是过度的运动运动以及认知和情感缺陷。HD是由亨廷顿蛋白（Htt）蛋白质中异常长的聚谷氨酰胺（polyQ）扩展引起的，该扩展会赋予突变型Htt毒性功能，导致神经变性。芦丁是一种黄酮类化合物，存在于植物，荞麦，一些茶和苹果中。尽管以前的研究已经表明，芦丁对HD模型具有一定的保护作用，但其潜在机制仍不清楚。在我们的研究中，我们调查了芦丁在HD秀丽隐杆线虫模型中的作用。我们评估了polyQ聚集，氧化损伤，神经变性水平和寿命，并研究了自噬和胰岛素/ IGF1（IIS）信号通路在芦丁诱导的有益作用中的可能作用。总体而言，我们的数据表明，慢性芦丁治疗减少了肌肉中的聚谷氨酰胺（polyQ）蛋白聚集，减少了ASH感觉神经元中polyQ介导的神经元死亡，并延长了寿命。涉及的可能机制是抗氧化剂活性，蛋白质降解的激活（自噬）和胰岛素/ IGF1（IIS）信号通路。这些发现表明，食用芦丁可能有助于预防HD，并且还提供了可能的途径，以探索针对与衰老相关的蛋白质病的新疗法。减少了ASH感觉神经元中polyQ介导的神经元死亡，并延长了寿命。涉及的可能机制是抗氧化剂活性，蛋白质降解的激活（自噬）和胰岛素/ IGF1（IIS）信号通路。这些发现表明，食用芦丁可能有助于预防HD，并且还提供了可能的途径，以探索针对与衰老相关的蛋白质病的新疗法。减少了ASH感觉神经元中polyQ介导的神经元死亡，并延长了寿命。涉及的可能机制是抗氧化剂活性，蛋白质降解的激活（自噬）和胰岛素/ IGF1（IIS）信号通路。这些发现表明，食用芦丁可能有助于预防HD，并且还提供了可能的途径，以探索针对与衰老相关的蛋白质病的新疗法。