发病机制
亨廷顿病
疾病
神经科学
线粒体
粒线体疾病
亨廷顿蛋白
透视图(图形)
生物
三核苷酸重复扩增
遗传学
生物信息学
医学
基因
线粒体DNA
病理
等位基因
免疫学
人工智能
计算机科学
作者
Yinghong Dai,Haonan Wang,Aojie Lian,Jinchen Li,Guihu Zhao,Shenghui Hu,Bin Li
出处
期刊:Mitochondrion
[Elsevier BV]
日期:2023-03-10
卷期号:70: 8-19
被引量:13
标识
DOI:10.1016/j.mito.2023.03.001
摘要
Huntington's disease (HD) is an autosomal dominant neurodegenerative disease. It is caused by the expansion of the CAG trinucleotide repeat sequence in the HTT gene. HD mainly manifests as involuntary dance-like movements and severe mental disorders. As it progresses, patients lose the ability to speak, think, and even swallow. Although the pathogenesis is unclear, studies have found that mitochondrial dysfunctions occupy an important position in the pathogenesis of HD. Based on the latest research advances, this review sorts out and discusses the role of mitochondrial dysfunction on HD in terms of bioenergetics, abnormal autophagy, and abnormal mitochondrial membranes. This review provides researchers with a more complete perspective on the mechanisms underlying the relationship between mitochondrial dysregulation and HD.
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