Better understanding of cystic fibrosis

Date:
October 6, 2021
Source:
University of Saskatchewan
Summary:
New understanding of cellular defects related to Cystic Fibrosis
(CF) could help pave the way for treatment.



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


==========================================================================
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 swine model of CF and used a specialized 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." Their findings were published
in the journal Cell Reports on Oct. 5.

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.

Ianowski has been working with Tam for about six years, and has been
able to meet people with CF in his clinic. He said this adds a sense of
purpose and desire to create applicable outcomes through the research
he performs in the lab.

"In this context I get to know the patients by name and see their
struggles," Ianowski said. "Working with Julian has created a meaningful partnership and we can inform and strengthen each other's work."
Tam and Ianowski point to the power of USask's rich research environment,
which gave them access to the expertise of veterinarians that supported
their work.

They also are grateful for the vital contributions of team members like
post- doctorate fellow Dr. Xianojie Luan (PhD), who played a crucial
role in developing the research protocols and collecting data.

The research was funded by the Canadian Institute for Health Research
and Cystic Fibrosis Canada.

========================================================================== Story Source: Materials provided by University_of_Saskatchewan. Note:
Content may be edited for style and length.


========================================================================== Journal Reference:
1. Xiaojie Luan, Yen Le, Santosh Jagadeeshan, Brendan Murray, James L.

Carmalt, Tanya Duke, Shannon Beazley, Masako Fujiyama, Kurtis
Swekla, Bridget Gray, Monique Burmester, Veronica A. Campanucci,
Alan Shipley, Terry E. Machen, Julian S. Tam, Juan P. Ianowski. cAMP
triggers Na absorption by distal airway surface epithelium in
cystic fibrosis swine.

Cell Reports, 2021; 37 (1): 109795 DOI: 10.1016/j.celrep.2021.109795 ==========================================================================

Link to news story: https://www.sciencedaily.com/releases/2021/10/211006160106.htm

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