背景:亨廷顿病是一种遗传性常染色体显性性状神经退行性疾病,由位于染色体短臂(p)的亨廷顿(htt)基因的变化(突变)引起4,CAG扩增突变。它的特点是不寻常的运动,认知和精神疾病。
目的:本综述旨在了解亨廷顿病(HD)发病机理的生物学途径及其通过自然衍生产品的管理。天然产品对于HD的管理可能是有利可图的,因为它在临床前试验中显示出对HD的保护作用。仍然需要先进的研究来评估已知有机产品及其分离化合物在HD实验模型中的治疗效果。
结论:亨廷顿病中神经元的变性表现为运动协调和肌肉功能的进行性丧失。这是由于在htt基因的第一个外显子中CAG三核苷酸的扩增导致大脑中的神经元死亡和神经元网络退化。人们认为,分子遗传学等因素,氧化应激,兴奋毒性,线粒体功能障碍,神经胶质功能障碍,蛋白质聚集,和改变UPS导致HD。天然产物的防御作用提供了针对HD的治疗功效。最近关于天然药物的报道已经启发了通过抗氧化剂对HD的保护作用,抗炎,抗凋亡,和神经功能调节。
BACKGROUND: Huntington\'s disease is an inherited autosomal dominant trait neuro-degenerative disorder caused by changes (mutations) of a gene called huntingtin (htt) that is located on the short arm (p) of chromosome 4, CAG expansion mutation. It is characterized by unusual movements, cognitive and psychiatric disorders.
OBJECTIVE: 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.
CONCLUSIONS: 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 htt 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.