Journal
MITOCHONDRION
Volume 70, Issue -, Pages 8-19Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.mito.2023.03.001
Keywords
Huntington?s disease; Mitochondrial dysfunction; Bioenergetics; Mitophagy; Mitochondrial Dynamics
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Huntington's disease (HD) is a neurodegenerative disorder caused by the expansion of the CAG trinucleotide repeat sequence in the HTT gene. It is characterized by involuntary dance-like movements and severe mental disorders. Mitochondrial dysfunction plays an important role in the pathogenesis of HD. This review discusses the role of mitochondrial dysfunction in HD in terms of bioenergetics, abnormal autophagy, and abnormal mitochondrial membranes, providing researchers with a more comprehensive understanding of the relationship between mitochondrial dysregulation and HD.
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 move-ments 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 mito-chondrial 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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