Protein involved in cystic fibrosis linked to chronic lung diseases
December 30th, 2010 - 3:19 pm ICT by ANIWashington, Dec 30 (ANI): A research team at Johns Hopkins Children’s Center has found that a protein involved in cystic fibrosis (CF) also regulates inflammation and cell death in emphysema and may be responsible for other chronic lung diseases.
The findings pave the way toward new treatments to prevent lung damage caused by infections or cigarette smoke in emphysema.
The protein, called CFTR (cystic fibrosis transmembrane conductance regulator), is already well known for its role in transporting chloride in and out of cells.
In CF, the protein’s chloride-carrying ability is absent due to genetic mutations, resulting in the buildup of thick sticky mucus in the lungs, which causes lung infections and breathing problems.
But the new Hopkins study indicates that CFTR is involved in immune regulation and immune response on a far wider scale.
The research - conducted in mice and using lung tissue from people with and without emphysema - shows that those with lung damage from emphysema had less CFTR on the cell surface and that changes in the level of CFTR corresponded directly to disease severity.
Decreases in CFTR also corresponded to increased buildup in the lung cells of a fatty molecule called ceramide, a well-known trigger of inflammation and cell death.
Thus, the researchers say, by regulating ceramide’s inflammation-causing activity, CFTR appears to be a watchdog for inflammation and cell death.
The study result has been published online in the December issue of The Journal of Immunology. (ANI)
- Scientists one step closer to a drug treatment for cystic fibrosis - Oct 13, 2010
- Novel drug may benefit cystic fibrosis patients - Dec 18, 2010
- Drug targets defective protein that causes cystic fibrosis - Nov 18, 2010
- Infliximab treatment effective for cystic fibrosis and Crohn's disease - Apr 20, 2010
- Crosstalk between ion channels leads to new therapeutic strategy - Sep 20, 2010
- Vitamin D may prevent or treat allergy to common mould - Aug 17, 2010
- Boffins come closer to understanding how cystic fibrosis causes lung disease - Apr 29, 2010
- Common cold virus may help treat cystic fibrosis - Jul 21, 2009
- Growth defects in patients with cystic fibrosis 'may start before birth' - Nov 10, 2010
- Scripps finding on DNA repair brings hope to cancer patients - Mar 28, 2011
- Inhalation therapy proves effective against cystic fibrosis - Feb 06, 2009
- New way to halt lung inflammation in animal models discovered - Jan 15, 2011
- Vitamin D may treat allergy to common mold - Aug 26, 2010
- New bacterial foe in cystic fibrosis identified - Oct 02, 2010
- Broccoli, cauliflower may help treat cystic fibrosis - Nov 17, 2009
Tags: breathing problems, cell death, cell surface, chronic lung diseases, cigarette smoke, cystic fibrosis, disease severity, emphysema, genetic mutations, hopkins study, immune regulation, immune response, lung cells, lung damage, lung infections, lung tissue, mucus in the lungs, s center, study result, thick sticky mucus