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U.K. Parliament Approves Controversial Three-Parent Mitochondrial Gene Therapy

The United Kingdom’s House of Commons voted overwhelmingly today to allow British researchers to pursue a new fertility treatment that could prevent certain kinds of genetic diseases. The technique, called mitochondrial DNA replacement therapy, could allow women who carry disease-causing mutations in their mitochondrial genes to give birth to genetically related children free of mitochondrial disease.

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The Ethics of Three Person IVF

The UK parliament will soon consider making Britain the first country to allow three person IVF. The regulations are yet to be approved, but the government is currently backing moves to allow the creation of babies with DNA from three people in cases where the children are at risk of inheriting mitochondrial disease.

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The Ethics of Three Person IVF

The UK parliament will soon consider making Britain the first country to allow three person IVF. The regulations are yet to be approved, but the government is currently backing moves to allow the creation of babies with DNA from three people in cases where the children are at risk of inheriting mitochondrial disease.

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New study shows how mitochondrial disease may be prevented

A joint team of scientists from The New York Stem Cell Foundation (NYSCF) Laboratory and Columbia University Medical Center (CUMC) has developed a technique that may prevent the inheritance of mitochondrial diseases in children. The study is published online today in Nature.

Dieter Egli, PhD, and Daniel Paull, PhD, of the NYSCF Laboratory with Mark Sauer, MD, and Michio Hirano, MD, of CUMC demonstrated how the nucleus of a cell can be successfully transferred between human egg cells. This landmark achievement carries significant implications for those children who have the potential to inherit mitochondrial diseases. Mitochondria are cellular organelles responsible for the maintenance and growth of a cell. They contain their own set of genes, passed from mother to child, and are inherited independently from the cell’s nucleus. Although mitochondrial DNA accounts for only 37 out of more than 20,000 genes in an individual, mutations to mitochondrial genes carry harmful effects.

Mitochondrial disorders, due to mutations in mitochondrial DNA, affect approximately 1 in 10,000 people, while nearly 1 in 200 individuals carries mutant mitochondrial DNA. Symptoms, manifesting most often in childhood, may lead to stunted growth, kidney disease, muscle weakness, neurological disorders, loss of vision and hearing, and respiratory problems, among others. Worldwide, a child is born with a mitochondrial disease approximately every 30 minutes, and there are currently no cures for these devastating diseases. “Through this study, we have shown that it should be possible to prevent the inheritance of mitochondrial disorders,” said Egli, PhD, co-author of the study and an Senior Researcher in the NYSCF Laboratory. “We hope that this technique can be advanced quickly toward the clinic where studies in humans can show how the use of this process could help to prevent mitochondrial disease.” In this study, the researchers removed the nucleus of an unfertilized egg cell and replaced it with the nucleus of another donor’s egg cell. The resultant egg cell contained the genome of the donor but not her mitochondrial DNA. The researchers demonstrated that the transfer did not have detectable adverse effects on the egg cell, a prerequisite for clinical translation. They achieved this by lowering the temperature of the egg before nuclear transfer, a novel technique. Previous studies report adverse consequences in approximately 50% of the egg cells. Read full article.

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Inheritance of Mitochondrial Disease Determined When Mother is an Embryo

(Medical Xpress)—The risk of a child to inherit mitochondrial diseases – i. e. malfunction in what is usually referred to as the power plants of the cell – is largely decided when the future mother herself is still an embryo. This according to a novel study by scientists at Karolinska Institutet and the Max Planck Institute in Germany, which is published in the journal Nature Genetics.

Mitochondria are small structures within almost every cell in the body, responsible mainly for energy production and fat metabolism. Their function is very important, and they contain their own genome, called mitochondrial DNA (mtDNA). The mitochondrial genome is inherited via the mother, where hundreds of thousands of mtDNA copies are packed in the female germ cell. Read full article.