Preventing Serious Mitochondria Disease

Every thirty minutes, a child is born who will be affected by mitochondrial diseases, a progressive wide-ranging group of disorders that arise due to mutations of nuclear or mitochondrial DNA, typically affecting the brain, muscles, nerves, the liver, the kidney and the heart. Often called the powerhouse of the cell, the mitochondria produce 90% of the energy that the body needs to function. The mitochondria’s main responsibility is to convert food and oxygen into life- sustaining energy in the form of adenosine triphosphate for cells to then use for specific functions. People with mitochondrial disease cannot make this conversion. Other than the nucleus, the mitochondria is the only part of the cell that has its own DNA. Mitochondrial DNA is transmitted to a child solely by its mother since paternal mitochondrial DNA is destroyed during fertilization
As a result, many mitochondrial diseases are contracted maternally with the result that any women with this disease pass mutated mitochondria directly down to their children. New treatments are now being developed that involve genetically modifying embryos in order to allow mothers with severe mitochondrial disease to give birth to healthy babies.
Currently the Assisted Human Reproduction Act (S.C. 2004, c2) prohibits (i) creating an embryo from a cell or part of a cell taken from an embryo or foetus for the purpose of creating a human being ; or (ii) transplanting an embryo so created into a human being. The question for consideration is whether genetic modification should be legalized for the purpose of preventing the transmission of mitochondrial disease?
At this time, no effective treatments of serious mitochondrial disease exist. Women with serious mitochondrial disease have the options of adoption or using a donor’s egg to conceive a healthy child. Scientists are currently investigating procedures of mitochondrial replacement to prevent transmission of mutated mitochondria from mother to child.