A study using ultra-deep mitochondrial DNA (mtDNA) sequencing has revealed an accumulation of mutations that affect oxidative phosphorylation in Huntington disease. The findings indicate that high levels of mutant huntingtin protein fragments result in increased mitochondrial fission and aberrant mitophagy, leading to a destabilization of mtDNA. The researchers suggest that enhancing mitochondrial health could offer a complementary therapy for Huntington disease.

中文翻译：

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亨廷顿病中的线粒体 DNA 不稳定性

一项使用超深线粒体 DNA (mtDNA) 测序的研究揭示了影响亨廷顿病氧化磷酸化的突变积累。研究结果表明，高水平的突变亨廷顿蛋白片段会导致线粒体裂变增加和异常线粒体自噬，从而导致线粒体 DNA 不稳定。研究人员认为，增强线粒体健康可以为亨廷顿病提供补充疗法。