May 9, 2024

Cystic Fibrosis Surprise – Scientists Discover Unexpected Function of Rare Lung Cells

CFTR Channels and Their Role.
CFTR channels that exist in respiratory tract secretory cells are understood to secrete chloride ions out of the cell and into the thin layer of liquid that covers the respiratory tract surface area. This respiratory tract surface area liquid plays an important role in defending the lungs versus damaging germs and particles. Since water “follows” salt, the outflow of chloride ions promotes hydration of the airway surface. On the other hand, the new study found that CFTR channels in ionocytes do the opposite; they take in chloride ions and promote wetness absorption..
” The crucial function that allows ionocytes to take in chloride is the ionocyte-specific barttin chloride channel on the opposite membrane of the cell from the CFTR channel,” states Ian Thornell, Ph.D., UI research assistant teacher of internal medication and senior author of the brand-new study released in the Oct. 16 problem of The Journal of Clinical Investigation. “Together, these two channels form a channel for chloride through the ionocyte that assists drain pipes the liquid lining the airways into the body.”.
Ramifications for Cystic Fibrosis.
The divergent roles of CFTR channels in these 2 different types of airway cells– ionocytes and secretory cells– likewise recommends that CF illness disrupts both liquid secretion and absorption, which might have implications for CF lung illness and for how CF drugs impact lung function. Because current CFTR modulator therapies bring back CFTR channel function, it is most likely that modulators deal with both secretion and absorption..
Recommendation: “CFTR-rich ionocytes mediate chloride absorption across air passage epithelia” by Lei Lei, Soumba Traore, Guillermo S. Romano Ibarra, Philip H. Karp, Tayyab Rehman, David K. Meyerholz, Joseph Zabner, David A. Stoltz, Patrick L. Sinn, Michael J. Welsh, Paul B. McCray and Ian M. Thornell, 16 October 2023, The Journal of Clinical Investigation.DOI: 10.1172/ JCI171268.
In addition to Thornell, the UI research study group included co-senior author Paul McCray Jr., MD, UI teacher of pediatrics-pulmonary medicine, and microbiology and immunology; and study very first author Lei Lei, PhD, UI postdoctoral scholar. UI researchers Soumba Traore; Guillermo Romano Ibarra; Philip Karp; Tayyab Rehman; David Meyerholz; Joseph Zabner; David Stoltz; Patrick Sinn; and Michael Welsh were likewise part of the research study group. The research study was funded in part by grants from the National Institutes of Health (DK054759, HL09184, HL147366, hl152960, and hl133089) and the Cystic Fibrosis Foundation..

CFTR channels that are present in respiratory tract secretory cells are known to produce chloride ions out of the cell and into the thin layer of liquid that covers the airway surface. This air passage surface area liquid plays a crucial function in protecting the lungs against damaging bacteria and particles. Since water “follows” salt, the outflow of chloride ions promotes hydration of the air passage surface area. UI researchers Soumba Traore; Guillermo Romano Ibarra; Philip Karp; Tayyab Rehman; David Meyerholz; Joseph Zabner; David Stoltz; Patrick Sinn; and Michael Welsh were also part of the research study group.

Illustration reveals lung ionocyte (pink) ingrained in air passage surface area (blue and yellow). Credit: Guillermo Romano Ibarra, University of Iowa
Researchers from the University of Iowa show that lung ionocytes play an essential function in the absorption of chloride and water.
Scientists from the University of Iowa have actually found that uncommon lung cells called pulmonary ionocytes assist in the absorption of water and salt from the respiratory tract surface. This function is exactly the opposite of what was expected of these cells and the findings could affect our understanding of cystic fibrosis (CF) lung illness.
Ionocytes in Human Lungs
5 years earlier, researchers reported the unanticipated discovery that ionocytes– a cell type commonly found in fish gills and frog skin– are likewise present in the lining of human lungs and respiratory tracts. These pulmonary ionocytes were especially intriguing to CF researchers since although they only account for about 1% of all the cells in the air passage lining, they consist of about half of the total amount of CFTR, the protein that is dysfunctional in cystic fibrosis..
Regardless of the ramification that CFTR-rich ionocytes might play an important role in CF, the function of these cells has actually remained unclear..