University of Calgary

Cutting-edge stem cell research takes aim at mitochondrial disease

UToday HomeMay 24, 2013

Principal investigator Jane Shearer, centre, gets ready to take part in the Scotiabank Calgary Marathon, along with several other members of her University of Calgary research team, from left: Kent Bates, Jason Myslicki, Christoper Newell, and Theresa Cowan.Principal investigator Jane Shearer, centre, gets ready to take part in the Scotiabank Calgary Marathon, along with several other members of her University of Calgary research team, from left: Kent Bates, Jason Myslicki, Christoper Newell, and Theresa Cowan.Faculty of Kinesiology researcher Jane Shearer, PhD, has received $50,000 in funding to advance research into a cutting-edge stem cell technology that may potentially lead to therapies to treat mitochondrial diseases. Working with three clinical experts in neurology and genetics from the Alberta Children’s Hospital, Shearer will examine the feasibility of using stem cells to treat these types of diseases.

Mitochondrial diseases are rare genetic disorders that directly impact the “power-plants” of the cell, the mitochondria. Mitochondria are key as they make energy for our bodies in the form of adenosine tri-phosphate, or ATP. There are over 200 disorders that can appear at birth or manifest later in life, affecting approximately one in 3,500 children. In many cases these diseases catastrophically change people’s lives.

The $50,000 funding for Shearer’s project – entitled Strategies to Enhance Stem Cell Mediated Mitochondrial Transfer– was provided by Mito Canada, a grassroots organization that raises awareness, supports families impacted by mitochondrial disease and funds research into treatment for mitochondrial disease. It was established by Blaine Penny, whose son Evan was diagnosed at the age of four with a form of mitochondrial disease that affects multiple systems in the body, including the brain, that rely heavily on ATP to function. Evan sustained brain damage and went from a healthy, active boy to being wheelchair-bound and unable to talk or perform even the most basic actions.

“The hope is, of course, that this innovative stem cell research may one day lead to new treatments that may lessen the impact of this terrible disease,” says Blaine Penny.

Shearer’s approach will essentially attempt to “transplant” mitochondria by injecting live human mesenchymalstem cells into an animal model. The experiment builds on previous research that has shown that these mitochondria will partially fuse with cells where the mitochondria have been removed or damaged. The goal of the project is to see how long the mitochondria transplant will last and how effective the transplanted mitochondria are in their new home. “It would only require a very small number of healthy mitochondria to be transferred to have a large therapeutic benefit for these children,” says Shearer.

Calgarians have an opportunity to find out more about mitochondrial disease and support Blaine Penny’s quest for a cure this weekend at the Scotiabank Calgary Marathon. Volunteers from the Mito Canada fundraising team will attempt to break the Guinness World Record for a “linked” marathon by running as a 10-person team tied by a rope. A five-person team from the United Kingdom currently holds the record with a time of 2:57:07 in October 2012.

The Mito Canada Foundation has raised over $500,000 in three years, some of which has supported ongoing university research projects.

 

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