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Abstract Mitochondrial genetic diseases are familial genetic diseases caused by the mutations of mitochondrial DNA (mtDNA), which seriously affect the physical and mental health of the patients. Mitochondrial replacement technology is applied to replace the mutant mtDNA in oocyte, and which is the important means to prevent mitochondrial diseases of the next generation. At present, mitochondrial replacement technology mainly includes cytoplasmic transplantation, pronuclear transfer, Spindle-chromosome complex transfer, and polar body transfer. These technologies have safety and ethical controversies, and which have not yet been introduced into clinical applications. This article focuses on summarizing the genetic characteristics of the mitochondrial genetic diseases, and provides a detailed introduction to the principles, advantages and disadvantages of four mitochondrial replacement technologies, as well as research progress in humans and animals. At the same time, the safety and ethical controversy of these technologies are reviewed for providing the theory references in the prevention and treatment of the mitochondrial genetic diseases using mitochondrial replacement technology.
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