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Image shows changes in airway cells at single-cell resolution. (Work by Janet Jiang and Zoltan Bozoky, Bear lab).

Christine Bear, senior scientist at The Hospital for Sick Children (SickKids), in collaboration with other labs, is working on treating cystic fibrosis-linked liver disease with regenerative medicine approaches.

Though cystic fibrosis is primarily seen as a lung disease, it is also a major cause of liver disease-related deaths when mucous build-up blocks the bile duct and damages its cells, which are called cholangiocytes. Eventually, this damage can destroy the liver.

For people living with these effects, transplantation is often the only hope – and two-thirds of those on transplant waiting lists don’t find a donor organ in time.

Bear, in collaboration with Shinichiro Ogawa, an affiliate scientist at the University Health Network’s McEwen Stem Cell Institute and assistant professor at the University of Toronto (U of T), has made progress generating cells that resemble mature cholangiocyte cells, offering potential treatments for liver disease in cystic fibrosis as well as other liver diseases.

Christine Bear

Christine Bear, senior scientist at The Hospital for Sick Children (SickKids). (Photo provided by SickKids.)

“These studies highlight the importance of generating mature cells from stem cells that faithfully mimic the functional properties of the native tissue,” says Bear, who is also a professor of physiology and biochemistry at U of T. “We can better understand the real effect of disease-causing mutations and cystic fibrosis therapies, especially in those hard-to-access organs.”

This work is supported by Medicine by Design, as is the larger team of liver-focused researchers led by Gordon Keller, director of the McEwen Stem Cell Institute.

Back to “Five ways Medicine by Design is transforming the treatment of liver disease.”