A comprehensive perspective of Huntington's disease and mitochondrial dysfunction.

Mitochondrion

National Clinical Research Center for Geriatrics Disorders, Department of Geriatrics, Xiangya Hospital, Central South University, Changsha, China. Electronic address:

Published: May 2023

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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http://dx.doi.org/10.1016/j.mito.2023.03.001DOI Listing

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