Regulation of gene expression in mitochondrial disease

线粒体疾病每年影响4000名儿童，其中大多数儿童没有达到成年期。这些疾病是由线粒体缺陷引起的，线粒体是细胞内产生能量的隔间。我们已经发现了一种控制线粒体基因表达的蛋白质，并将其用于挽救线粒体疾病患者的细胞功能障碍。我们将确定其在细胞健康中的功能和作用，使我们能够评估其在线粒体疾病中的重要性。

Mitochondrial diseases affect 4000 children every year and most of these children do not reach adulthood. These diseases result from defects in mitochondria, energy producing compartments within cells. We have discovered a protein that controls mitochondrial gene expression and used it to rescue dysfunction in cells from patients with mitochondrial disease. We will determine its function and role in cell health, allowing us to evaluate its importance in mitochondrial disease.