Cystic fibrosis is a disease of the lungs which is caused by a malfunction of the CFTR gene. The said gene codes for the channel or transporter of chloride and water across the semi-permeable membrane of the cell. The most prominent clinical manifestation of this disorder is salty sweat, and is always checked to help diagnose the disease. People affected with this disorder have excessive production of thick and sticky mucus which consequently clog the airways. The clogged airways provide the perfect medium of growth for several bacterial pathogens.
One of the bacteria that commonly inhabit the airways in cystic fibrosis is Burkholderia cenocepacia. This organism is a member of the Burkholderia cepacia group of bacteria. It is commonly isolated from soil and water. Although an estimated percentage of only 2 to 5 percent of patients with CF are shown to be infected with this group of bacteria, the latter is highly resistant to antibiotics making it a difficult target for therapeutic agents.
In a recent biomedical science research published in the May issue of PLoS One, it was concluded that the use of IFN-γ as an immune booster by activating a natural cell death process called autophagy, has shown to be effective in the eradication of this lethal bacterial infection in cystic fibrosis.
Dr. Benjamin T. Kopp, senior author of the study, principal investigator for the Center for Microbial Pathogenesis, pulmonologist at Nationwide Children’s and assistant professor of pediatrics at The Ohio State University College of Medicine, explained that the multi-drug resistant property of B. cenocepacia imposes cocktail medication therapy on patients and physicians can only hope for the favorable effects of the combined drugs to take effect.
IFN-γ, which is produced by the IFNG gene found in humans, is a stimulator of the immune system. The cytokine was previously shown in past studies to have the capability of stimulating autophagy. In diseases such as cystic fibrosis, this process of natural death and cell degradation was found to be inhibited.
The study was done by isolating macrophages from patients who were affected with cystic fibrosis (CF) and from those who were not. The macrophages where then cultured and manually infected with B. cenocepacia. Studies of the macrophages from CF patients showed decreased levels of IFN-γ. The macrophages were then treated with synthetic IFN-γ, which was found to enhance autophagy in the cultured cells and decreased the colonization of the bacteria.
The objective of their study, Kopp explained, is to prove that the administration of IFN-γ can indeed stimulate autophagy and aid in the eradication of pathogens. And with the results of the study, they have successfully proven their theory and have added yet another contribution to biomedical science innovations.