14 DAY TRIAL // According to the results of a new scientific investigation, it would appear that a protein which plays an important role in underlying cystic fibrosis (CF) is also heavily involved in the development of chronic lung diseases, such as emphysema.
The molecule apparently regulates cellular processes such as inflammation and death in this particular condition, say researchers at the Johns Hopkins Children’s Center.
Scientists say that the research paves the way for the development of new drugs and therapies, that could be directed towards preventing lung damage that is oftentimes caused during emphysema by cigarette smoke and certain infections.
Details of the structure and functions of the CFTR (cystic fibrosis transmembrane conductance regulator) protein are published in the December online issue of the Journal of Immunology.
In past studies, researchers established that one of the main functions of this particular molecules was to set the foundation for transporting the chemical chloride in and out of the cell, Science Blog reports.
Genetic mutations can cause the protein's transport ability to disappear, and this is one of the reasons why CF develops. The lungs start to accumulate a thick, sticky, mucus-like substance, which makes it harder to breathe, and can lead to severe infections.
But the new research manged to demonstrate that, in fact, the CFTR protein plays a much more important and widespread role in the body's immune regulation and immune response than believed.
During the experiments they conducted, the experts used unsuspecting mice for investigating the function of the protein. Additionally, they also used lung tissues from people who either had, or did not have, emphysema.
It was determined that patients who suffered from the condition had far lower concentrations of CFTR on the surface of their lung cells than healthy patients. A causal link was also found between low protein concentrations and the severity of CF in that particular individual.
“Our findings suggest that CFTR is a multi-tasker protein that is not only involved in chloride transport but also in regulating cell death and inflammation by keeping in check the rampant and dangerous accumulation of [a fatty molecules called] ceramide ,” explains Neeraj Vij, PhD.
The scientist was the principal investigator on the study. He holds an appointment as a pulmonary researcher at Hopkins Children’s, and is also an assistant professor at the Johns Hopkins University (JHU) School of Medicine.
“We anticipate that membrane CFTR and ceramide may turn out to be useful predictors of susceptibility to lung damage from smoking and infections and may be tailored for drug therapy to alter disease course,” he concludes.