Researchers at the University of Saskatchewan (USask) are hopeful that a new understanding of cellular defects related to Cystic Fibrosis (CF) could help pave the way for the treatment of the disease.

Their findings of the study were published in the journal Cell Reports. A team in the College of Medicine led by Drs Juan Ianowski (PhD) and Julian Tam (MD) found that sodium transport is abnormal in lungs with CF. The researchers, affiliated with the Respiratory Research Centre, studied the model of CF and used a specialised microelectrode technique that allowed them to perform experiments with very high resolution.

They discovered there is excessive sodium absorption in the small airways, a previously unstudied site in the body. "A precise understanding of the cellular basis of CF lung disease is a prerequisite for the development of treatments such as gene therapy that have the potential to cure CF," said Tam. "CFTR modulators, such as Trikafta, can improve life for about 90 per cent of patients. Our work is especially relevant to that 10 per cent of people with CF who cannot benefit from these medications," added Tam.

According to Cystic Fibrosis Canada, CF is the most common fatal genetic disease affecting Canadian children and young adults. There is presently no cure for the disorder that varies in symptoms from patient to patient, but mainly affects the lungs and digestive system. About one in every 3,600 children born in Canada has CF, which occurs when a person receives two copies of a defective gene -- one from each parent.