Airway Surface Liquid or ASL could be key to treating CF

E

enniob

Administrator
Staff member
A SIGNIFICANT BREAKTHROUGH has occurred from scientist studying pigs tracheas that have been modified to mimic CF lungs. They discovered that CF lungs do not produce a very important and almost undetectable thin layer of fluid that lines the lungs, called "airway surface liquid" (ASL for short). This liquid and gelatin substance is thought to trap inhaled particles and bacteria, and kill micro-organisms before little hairs called cilia sweep the mucus out of the lungs.

Now that we know this, drugs can be designed to stimulate the lungs to produce this ASL lining, which may significantly reduce the chance and frequency of life threatening lung infections in CF patients. This may have a significant impact of life expectancy since lung infection is one of the leading and potentially fatal complications in CF patients.

Prof. Juan Ianowski said: "I believe that what we are trying to study may be essential, and may be the key (to the disease),". Ianowski's research is part of a "paradigm shift" in cystic fibrosis research, which is the ability to study the disease in large animals like pigs.

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- Chris
 
E

enniob

Administrator
Staff member
Hi Werner, this is a nice article explaining how/why pigs are used to study CF. http://www.sciencedaily.com/releases/2011/03/110316152947.htm
In 2008, the UI team and colleagues at University of Missouri created pigs that were missing the CFTR protein. These animals developed CF disease symptoms that closely mimicked the human disease. In the new pig model, the animals have two copies of the CFTR gene containing the most common CF-causing mutation, which is known as the delta F508 mutation. These pigs also develop CF symptoms similar to the human disease. In particular, the CF pigs are born with gastrointestinal disease and develop lung disease over time.
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-chris
 
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