Radio Shows | Biofilms and Cystic Fibrosis | mp3 … wma … wav
Imagine your average life span being in your 30's. That's the fate with certain genetic diseases like Cystic Fibrosis or CF.
Fortunately research is working to change that.
If you don't know anything about CF, it's an inherited disease that affects the lungs and digestive tract and there's no cure. Across the population there is about a one in fifty chance of having a mutated gene.
The mutated gene in CF is the cystic fibrosis transmembrane conductance regulator. This gene encodes a protein that helps to control the movement of salt or sodium chloride in cells.
The mutation causes the protein to misfold which is then destroyed by the cell. Without the protein to remove salt, the cell compensates and begins to pull water in, leaving little fluid outside the cell. That in turn creates the thick, sticky mucus found in CF patients.
Mucus is a slippery substance that protects the lining of the airways, digestive system, reproductive system and other organs and tissues.
Without water to thin the mucus, it thickens and ends up blocking the lungs making it not only hard to breathe but provides ideal conditions for bacteria which can cause pneumonia.
The leading cause of death in CF patients is an infection caused by the bacterium Pseudomonas aeruginosa. It produces something called phenazines which stains the mucus blue-green.
For decades scientists thought the bacterium generated phenazines in order to kill off competing bacteria in the lungs. Now a study shows phenazines create a smooth biofilm surface under which pseudomonas aeruginosa can grow and thrive.
Researchers hope by developing antibiotics that target phenazines, Pseudomonas aeruginosa will have a harder time thriving which will give CF patients a better chance at life.
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