Nitric Oxide Chemistry Contributes To Cystic Fibrosis Research

There may be new hope for improved treatments and longer life for cystic fibrosis patients, thanks to a recent discovery by a team of researchers including Brookhaven National Laboratory chemist Sergei Lymar. Led by University of Cincinnati microbiologist Daniel Hassett, the team has discovered what might be the “Achilles heel” of a dangerous organism that lives in the lungs of cystic fibrosis patients—a fatal flaw that leaves the organism vulnerable to destruction by a common food preservative.

“Treatment for cystic fibrosis consists of clearing the patient’s lungs of mucus, a stagnant medium where opportunistic bacteria thrive,” Lymar said. “It is difficult to reach the bacteria there with antibiotics, but nitric oxide may be the key to an important new therapy.”

Cystic fibrosis, which affects about 30,000 people in the United States, is an inherited disease caused by a defect in a single gene in a chromosome of both parents. Affecting the lungs and many other vital organs, cystic fibrosis is chronic, progressive, and ultimately fatal, mostly as a result of serious lung infections that breed in the thick and sticky mucus caused by the abnormal gene. Until the 1980s, most deaths from cystic fibrosis occurred in children and teenagers. Today, thanks to improved treatments, people with cystic fibrosis live an average of 35 years.

It has been known for some time that an opportunistic pathogen, Pseudomonas aeruginosa, a common inhabitant of soil and water, invades the lung-clogging mucus found in the airways of cystic fibrosis patients and significantly weakens them. In the lungs, Pseudomonas mutates to form a dense, resistant biofilm that leads to lung destruction. The new study suggests that this mutation harbors a fatal flaw that could help physicians clear the characteristic “goop” from the lungs of advanced cystic fibrosis patients. The same genetic change that turns the organism into an antibiotic- and immune-system resistant killer also leaves it susceptible to destruction by slightly acidified sodium nitrite, a common chemical that is widely used in the curing of lunch meat, sausages, and bacon.