Scientists discover basic defect in cystic fibrosis airway glands ( Scientists at Stanford University have ...)

Scientists discover basic defect in cystic fibrosis airway glands

Scientists at Stanford University have determined that the buildup of sticky mucus found in cystic fibrosis is caused by a loss in the epithelial cell's ability to secrete fluid. This research appears as the "Paper of the Week" in the March 17 issue of the Journal of Biological Chemistry, an American Society for Biochemistry and Molecular Biology journal.

Cystic fibrosis is the most common, fatal genetic disease in the United States. It causes the body to produce thick, sticky mucus that builds up in the lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated serious lung infections. The thick, sticky mucus can also block tubes in the pancreas, preventing digestive enzymes from reaching the small intestine.

The disorder results from mutations in the gene for the cystic fibrosis transmembrane conductance regulator (CFTR), a membrane channel regulator essential for proper salt and water movement across some epithelia. Currently, there are two essentially opposite explanations for the inability of the body to clear mucus from the airways in cystic fibrosis. The first is that the defective CFTR is unable to aid in fluid secretion in cystic fibrosis airway glands. The second explanation is that the glands still secrete fluid via non-CFTR pathways, but the fluid is reabsorbed by other channels. In fact, it has been proposed that one of CFTR's functions is to inhibit the activity of a channel called the epithelial Na+ channel (ENaC).

Nam Soo Joo and colleagues at Stanford University attempted to determine which hypothesis was correct by measuring the secretion from glands from patients with cystic fibrosis and from normal pigs. They added ENaC inhibitors to the glands to determine if the channel plays a role in mucus clearance. The researchers found no evidence that the inhibitors altered secretion rates in either normal or cystic fibrosis glands. This suggested that loss of CFTR-mediated fluid secretion
'"/>

Source:American Society for Biochemistry and Molecular Biology

Page: 1 2
Related biology news :

1. Scientists ID molecular switch in liver that triggers harmful effects of saturated and trans fats
2. Scientists Replicate Hepatitis C Virus in Laboratory
3. Scientists detect probable genetic cause of some Parkinsons disease cases
4. Scientists find missing enzyme for tuberculosis iron scavenging pathway
5. Scientists seek answers on what activates deadly anthrax spores
6. Yale Scientists Find MicroRNA Regulates Ras Cancer Gene
7. Scientists collaborate to assess health of global environment
8. Scientists decipher genome of fungus that can cause life-threatening infections
9. Scientists discover the cellular roots of graying hair
10. Scientists rid stem cell culture of key animal cells
11. Scientists develop new color-coded test for protein folding

Post Your Comments:

( Scientists at Stanford University have ...)Scientists discover basic defect in cystic fibrosis airway glands