转线粒体小鼠的研究进展

线粒体转移技术的发展和成熟使得我们业已成功的建立了转线粒体小鼠动物模型。目前常用的方法主要有:一是直接应用显微注射技术将活的线粒体转入小鼠胚胎;二是通过脱核胞质体与胚胎干细胞融合,再将胚胎干细胞显微注入小鼠囊胚,从而形成嵌和鼠;三是将脱核胞质体与小鼠胚胎直接融合而产生的转线粒体小鼠。随着越来越多线粒体相关疾病的发现,各种不同线粒体疾病的转线粒体小鼠的开发具有十分重要的应用价值和广阔的研究前景。

Advances in Research on Transmitochondrial Mouse Model

With the development of transmitochondrial technique,we have successfully establisheda a transmitochondrial mouse model carrying mutated mitochondrial DNA.Now there are three methods to produce transmitochondrial mice.One is direct mitochondrial microinjection.The second is by fusing cytoplasts produced by enucleation of cells(donor) derived from patients or controls with undifferentiated female mouse embryonic stem cells and then injecting the embryonic stem cells into blastocysts.The third is direct by fusing cytoplasts with one-cell mouse embryo.With the discovery of a number of mitochondrial related diseases,tramsmitochondrial mice containing human mitochondrial disorders will have a great value to investigate mtDNA mutation and mitochondrial defect pathogenesis,evaluate gene therapy of diseases caused by these mutations and screen drugs of treating mitochondrial diseases.