Abstract
The Cytoplasm of mammalian oocytes contains enormous amounts of mitochondria/mitochondrial DNA (mtDNA), which is important for mammalian fertilization and subsequent early embryonic development. MtDNA inheritance is exclusively maternal and only passes from mother to child through eggs. Owing to this unique feature of mitochondrial inheritance, it is feasible to replace mitochondria/mtDNA via innovate technology, which is called mitochondrial replacement therapy1). We developed the maternal spindle transfer (MST) using primate mature MII oocytes and demonstrated efficient replacement of mitochondria/mtDNA. Furthermore, we demonstrated that mtDNA can also be efficiently replaced in humans. With the advent of reliable and safer technologies, several MRT techniques, including MST, are now considered for germline gene therapy for inherited mitochondrial diseases. Recently, several clinical trials are underway. In this review, the utilization of MRT for germline gene therapy of inherited mitochondrial diseases, as well as its potential applications in standard ART practice, are described.
