Irfan Zainab, Khanam Sofia, Karmakar Varnita, Firdous Sayeed Mohammed, El Khier Bothaina Samih Ismail Abou, Khan Ilyas, Rehman Muneeb U, Khan Andleeb
Department of Pharmaceutical Technology, Brainware University, Kolkata 700125, West Bengal, India.
Department of Pharmacology, Calcutta Institute of Pharmaceutical Technology & AHS, Howrah 711316, West Bengal, India.
Brain Sci. 2022 Oct 14;12(10):1389. doi: 10.3390/brainsci12101389.
Huntington's disease is an inherited autosomal dominant trait neuro-degenerative disorder caused by changes (mutations) of a gene called huntingtin () that is located on the short arm (p) of chromosome 4, CAG expansion mutation. It is characterized by unusual movements, cognitive and psychiatric disorders.
This review was undertaken to apprehend biological pathways of Huntington's disease (HD) pathogenesis and its management by nature-derived products. Natural products can be lucrative for the management of HD as it shows protection against HD in pre-clinical trials. Advanced research is still required to assess the therapeutic effectiveness of the known organic products and their isolated compounds in HD experimental models.
Degeneration of neurons in Huntington's disease is distinguished by progressive loss of motor coordination and muscle function. This is due to the expansion of CAG trinucleotide in the first exon of the gene responsible for neuronal death and neuronal network degeneration in the brain. It is believed that the factors such as molecular genetics, oxidative stress, excitotoxicity, mitochondrial dysfunction, neuroglia dysfunction, protein aggregation, and altered UPS leads to HD. The defensive effect of the natural product provides therapeutic efficacy against HD. Recent reports on natural drugs have enlightened the protective role against HD via antioxidant, anti-inflammatory, antiapoptotic, and neurofunctional regulation.
亨廷顿舞蹈症是一种常染色体显性遗传的神经退行性疾病，由位于4号染色体短臂（p）上的亨廷顿基因（HTT）发生变化（突变），即CAG重复序列扩增突变引起。其特征为异常运动、认知和精神障碍。
本综述旨在了解亨廷顿舞蹈症（HD）发病机制的生物学途径及其天然产物治疗方法。天然产物对HD的治疗可能有益，因为在临床前试验中显示出对HD的保护作用。仍需进一步研究来评估已知有机产物及其分离化合物在HD实验模型中的治疗效果。
亨廷顿舞蹈症中神经元的退化表现为运动协调和肌肉功能的逐渐丧失。这是由于负责神经元死亡和大脑神经网络退化的HTT基因第一外显子中CAG三核苷酸的扩增。据信，分子遗传学、氧化应激、兴奋性毒性、线粒体功能障碍、神经胶质细胞功能障碍、蛋白质聚集和UPS改变等因素会导致HD。天然产物的保护作用为HD提供了治疗效果。最近关于天然药物的报道揭示了其通过抗氧化、抗炎、抗凋亡和神经功能调节对HD的保护作用。
