Treating Infections in Cystic Fibrosis Patients

Reducing levels of a protein called p62 may help reduce the risk of fatalities due to cystic fibrosis, researchers say. Suppressing the protein in the immune systems of experimental mice was found to trigger a process that ultimately results in the elimination of a bacterial infection that commonly strikes—and often kills—people with the hereditary condition.

The gene that causes cystic fibrosis has the effect of creating sticky mucous in the lungs and the digestive system, affecting the functioning of both. The mucous is supposed to function as a lubricant, but in cystic fibrosis patients, it instead does the opposite. It is a recessive disease: one in four children of two carriers, and half the children of one carrier and one patient, will have the condition.

Cystic fibrosis leads to a number of complications. One of these is a type of lung dysfunction called bronchiectasis. Patients also tend to cough up blood. The condition makes people prone to diabetes, nasal polyps, and respiratory infections. It also affects reproductive health, rendering men completely infertile and affecting, though in a lesser degree, the fertility of women. Pregnancy, however, can exacerbate the condition.

One common infection associated with cystic fibrosis is called cepacia syndrome, a frequently fatal infection that is caused by a bacterium called Burkholderia cenocepacia and that leads to fever and pneumonia with weight loss. Cepacia disrupts the process by which the immune system destroys infective cells. Although the bacteria itself is invulnerable to antibiotics, a medication called rapamycin that stimulates that process might be able to control the effects of the infections.

Rapamycin, however, has serious side effects, including general immune suppression and the diabetes precursor insulin resistance. Researchers are looking at altering the production of the protein p62 as another approach. The protein is sticky and causes clustering that slows down the clean-up process, so reducing levels of it speeds it back up.

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