Mitochondria are the nexus of cellular energy metabolism and major signaling hubs that integrate information from within and without the cell to implement cell function. Mitochondria harbor a distinct polyploid genome, mitochondrial DNA (mtDNA), that encodes respiratory chain components required for energy production. MtDNA mutation and depletion have been linked to obesity and metabolic syndrome in humans. At the cellular and subcellular levels, mtDNA synthesis is coordinated by membrane contact sites implicated in lipid transfer from the endoplasmic reticulum, tying genome maintenance to lipid storage and homeostasis. Here, we examine the relationship between mtDNA and lipid trafficking, the influence of lipotoxicity on mtDNA integrity, and how lipid metabolism may be disrupted in primary mtDNA disease.

中文翻译：

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连接脂质代谢和线粒体基因组维护


线粒体是细胞能量代谢的纽带，也是整合细胞内外信息以实现细胞功能的主要信号传导中枢。线粒体拥有独特的多倍体基因组，即线粒体 DNA (mtDNA)，它编码能量产生所需的呼吸链成分。 MtDNA 突变和耗竭与人类肥胖和代谢综合征有关。在细胞和亚细胞水平上，mtDNA 合成由参与内质网脂质转移的膜接触位点协调，将基因组维护与脂质储存和稳态联系起来。在这里，我们研究了线粒体DNA和脂质运输之间的关系、脂毒性对线粒体DNA完整性的影响，以及原发性线粒体DNA疾病中脂质代谢如何被破坏。