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CF milestones at UI and beyond

CF timeline

1985 Discovery that cystic fibrosis (CF) disrupts chloride transport across airway epithelium, and the defect is localized to the apical (air-side) surface of the cells (PNAS).
1988 University of Iowa Cystic Fibrosis Research Center established.
1989 Cystic fibrosis transmembrane conductance regulator (CFTR) gene discovered.


UI team finds expressing recombinant CFTR in CF cells corrects CF chloride defect (Nature).
1991 UI team determines CFTR is a chloride channel (Science). 
1992 UI researchers discover that CF airway cells fail to secrete bicarbonate ions (J. Clin. Invest.)

Beginning of National Institutes of Health funding for UI CF researchers; more than $80 million awarded to date. 

UI team develops classification system describing how different genetic mutations can “break” the CFTR protein (Cell). System still used today, has guided development of therapies to repair the breaks.

UI launches first human trials of gene therapy for CF (Cell).

1998 UI provides Vertex Pharmaceuticals with cells expressing functional and mutated CFTR, enabling high-throughput screening of drugs to correct CF defect.
2008 Pig model of CF developed by UI and University of Missouri teams (Science). 
2010 Ferret model of CF developed at UI (J. Clin. Invest.).

Kalydeco approved by Food and Drug Administration to treat certain forms of CF; tested at UI.

UI determines airway surface liquid more acidic in CF lungs, suggesting new treatment possibilities (Nature).

2014 UI team shows mucociliary transport defective in CF at birth (Science).
2016 UI researchers discover a proton pump, ATP12A, secretes acid into airways of people and pigs with CF; experimentally disabling ATP12A in pigs with CF halts development of lung infection (Science).


Back to the state of UI cystic fibrosis research

Medical illustration of lungs for Medicine Iowa magazine cover

Featured in Medicine Iowa Spring 2018

You’re reading one of the features included in the Spring 2018 issue of Medicine Iowa. Read more news and features about the people and programs focused on teaching, healing, and research in the UI Roy J. and Lucille A. Carver College of Medicine.