Legge Michael, Fitzgerald Ruth
Department of Biochemistry, University of Otago, Dunedin, New Zealand.
N Z Med J. 2013 Nov 1;126(1385):71-5.
Inherited mitochondrial disorders affect between 1 in 5000 to 1 in 8000 people. These are a heterogeneous group of maternally-inherited disorders, with an array of outcomes such as heart and liver failure, defects in energy metabolism, blindness, deafness, loss of motor skills and premature death. Recently the Human Fertilisation and Embryology Authority provided advice to the UK Government to permit the use of enucleated donated oocytes with normal (wild-type) mitochondria (a currently prohibited IVF technique) to be used as recipients of nuclear DNA from intending mothers to overcome transmission of mitochondrial disorders. In this short communication we present the basis for this radical new IVF technology, and discuss the implications for its use both in the context of treating a group of inherited disorders and the current New Zealand IVF legislation.
遗传性线粒体疾病影响着五千分之一到八千分之一的人群。这些是一组异质性的母系遗传疾病，会产生一系列后果，如心力衰竭、肝功能衰竭、能量代谢缺陷、失明、失聪、运动技能丧失和过早死亡。最近，人类受精与胚胎学管理局向英国政府提供建议，允许使用带有正常（野生型）线粒体的去核捐赠卵母细胞（一种目前被禁止的体外受精技术）作为意向母亲核DNA的受体，以克服线粒体疾病的遗传。在这篇简短的通讯中，我们阐述了这项全新体外受精技术的依据，并讨论了其在治疗一组遗传性疾病以及新西兰现行体外受精立法背景下的应用意义。
