New data supports ongoing clinical trials and raise the possibility of new targets for the development of drugs to treat individuals with CF, according to the Journal of Clinical Investigation.
The genetic mutations in CF patients cause their lung cells to make a compartment of the cell known as the TGN more acidic than it is in cells from healthy individuals. In the study led by Vojo Deretic and colleagues at the University of New Mexico School of Medicine, Albuquerque, in vitro analysis revealed that hyperacidification of the TGN causes increased activity of a protein known as furin.
Through increased production of a soluble factor known as TGF-beta, this augmented the production of collagen (which is associated with tissue fibrosis), suppressing their ability to fight the Pseudomonas aeruginosa bacterium — a major complication for individuals with CF.
This study provides strong support for the use of chloroquine (which counteracts high levels of TGN acidity) to treat CF, something that is currently being tested in clinical trials, and identifies furin inhibitors as potential new therapeutics for the treatment of CF.
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