Bioinformatics Lab, Department of Biotechnology, School of Bio Sciences and Technology, VIT (Deemed to be University), Vellore, 632014, Tamil Nadu, India.
Bioinformatics Lab, Department of Biotechnology, School of Bio Sciences and Technology, VIT (Deemed to be University), Vellore, 632014, Tamil Nadu, India.
Prog Biophys Mol Biol. 2019 Aug;145:40-51. doi: 10.1016/j.pbiomolbio.2018.12.003. Epub 2018 Dec 10.
Amyotrophic lateral sclerosis (ALS) is a calamitous neurodegenerative disorder characterized by denervation of upper and lower motor neurons. Numerous hypotheses suggest that toxic protein misfolding and aggregation cause ALS, similar to that of other neurodegenerative diseases, such as Alzheimers and Parkinsons. Protruding causes of familial ALS are mutations in the gene encoding Cu/Zn superoxide dismutase-1 (SOD1), which decrease protein stability and endorse protein aggregation. Thus, the interference concerning aggregate formation and destabilization in SOD1 is considered to be an impending therapeutic strategy. In this work, we utilized computational chemistry methods to initially study the effect of substitution mutation, His46Arg on SOD1 protein. Further, we described the interaction of two naturally occurring polyphenol compounds, naringin and naringenin with mutant SOD1 that is regarded to hinder the protein aggregation. Subsequently, the use of quantum chemical and molecular mechanics calculations speculated that naringin had a strong binding affinity with mutant SOD1 and impeded the formation of toxic aggregates than that of naringenin. Ultimately, we could conjecture that ingesting of polyphenol-rich foods in ALS patients may be regarded to improvise their living. Moreover, the findings from our study could pave a way in the field of structure-based drug design in developing potential anti-aggregation inhibitors against incurable ALS, affecting the human population.
肌萎缩侧索硬化症(ALS)是一种灾难性的神经退行性疾病,其特征是上下运动神经元的去神经支配。许多假说表明,有毒蛋白质错误折叠和聚集导致 ALS,类似于其他神经退行性疾病,如阿尔茨海默病和帕金森病。家族性 ALS 的突出原因是编码铜/锌超氧化物歧化酶-1(SOD1)的基因突变,这会降低蛋白质稳定性并促进蛋白质聚集。因此,干扰 SOD1 中聚集体形成和失稳被认为是一种有前途的治疗策略。在这项工作中,我们利用计算化学方法初步研究了取代突变 His46Arg 对 SOD1 蛋白的影响。此外,我们描述了两种天然多酚化合物柚皮苷和柚皮素与突变 SOD1 的相互作用,这被认为可以阻止蛋白质聚集。随后,量子化学和分子力学计算推测,柚皮苷与突变 SOD1 具有很强的结合亲和力,并能阻止有毒聚集体的形成,其效果强于柚皮素。最终,我们可以推断,富含多酚的食物在 ALS 患者中的摄入可能被认为可以改善他们的生活。此外,我们的研究结果可能为基于结构的药物设计领域开辟道路,以开发针对无法治愈的 ALS 的潜在抗聚集抑制剂,影响人类。