Genetic Engineering Laboratory, Indian Institute of Chemical Biology (C.S.I.R), 4 Raja S. C. Mullick Road, Calcutta 700032, India.
Mitochondrion. 2011 Jul;11(4):607-14. doi: 10.1016/j.mito.2011.03.124. Epub 2011 Apr 6.
Many human diseases are associated with mutations and deletions in mitochondrial DNA (mtDNA). We have generated a cell line, EB delta1, with multiple mtDNA deletions, that is respiration-defective and generates high levels of superoxide, a reactive oxygen species. Treatment of EB delta1 with tagged polycistronic (pc) RNAs, encoding parts of the mitochondrial proteome, bound to a multi-subunit carrier complex, resulted in cellular uptake and transfer of the RNA to mitochondria, restoration of respiration, and suppression of superoxide levels. These findings have implications for correction of mitochondrial defects in age-related disorders due to mtDNA mutations.
许多人类疾病都与线粒体 DNA（mtDNA）的突变和缺失有关。我们生成了一个具有多种 mtDNA 缺失的细胞系 EB delta1，该细胞系呼吸缺陷并产生高水平的超氧化物，这是一种活性氧。用标记的多顺反子（pc）RNA 处理 EB delta1，这些 RNA 编码线粒体蛋白质组的一部分，与多亚基载体复合物结合，导致 RNA 被细胞摄取并转移到线粒体，呼吸恢复，超氧化物水平降低。这些发现对因 mtDNA 突变导致的与年龄相关疾病中的线粒体缺陷的纠正具有重要意义。
