cystic fibrosis

Females with cystic fibrosis have more severe disease than males with cystic fibrosis and have a shorter lifespan.

Although many suggestions have been put forward to explain this sex-related difference, a concrete mechanism to explain it has remained elusive.

Cystic fibrosis continues to be a lethal disease for humans despite the identification of the problematic gene two decades ago.

Many humans born with CF -- the most common genetic disease in Caucasians -- often die because of a lung disease developed later.

Scientists have been unable to develop an animal model that develops the fatal lung disease. Now, one researcher is producing pigs born with cystic fibrosis that mimic the exact symptoms of a newborn with CF.

(University of Bristol) A new drug therapy may represent a tremendous step forward in the treatment of some 70,000 cystic fibrosis patients worldwide, Dr.

David Sheppard from the University of Bristol will tell an audience at the BA Festival of Science in Liverpool tomorrow.Speaking ahead of the conference, Dr.

Sheppard said:"The early results with VX-770 suggest that drug therapies which target defects at the root of the disease have the potential to improve greatly the quality of life of CF patients."

By manipulating the machinery used by our cells for quality control, researchers have found a way to restore the function of cystic fibrosis (CF) airway cells.

This could significantly reduce the sticky mucus that plugs the lungs of CF patients, which leads to antibiotic-resistant infections and untimely death.

(Federation of American Societies for Experimental Biology) By manipulating the machinery used by our cells for quality control, researchers from the University of Pittsburgh have found a way to restore the function of cystic fibrosis (CF) airway cells.

This could significantly reduce the sticky mucus that plugs the lungs of CF patients, which leads to antibiotic-resistant infections and untimely death.

The study appears in the September 2008 print issue of the FASEB Journal.

Chemical engineers have broken the "mucus barrier," engineering the first drug-delivery particles capable of passing through human mucus -- regarded by many as nearly impenetrable -- and carrying medication that could treat a range of diseases.

Those conditions include lung cancer, cervical cancer and cystic fibrosis, the research say.

Researchers have discovered a key component of the quality control mechanism that operates inside human cells -- sometimes too well.

The breakthrough has significant implications for the development of new treatments for cystic fibrosis and some other hereditary diseases, the researchers say.

(McGill University) Researchers in Japan and Canada have discovered a key component of the quality control mechanism that operates inside human cells -- sometimes too well.

The breakthrough has significant implications for the development of new treatments for cystic fibrosis and some other hereditary diseases, the researchers say.

Their results were published July 25 in the journal Science.

(University of Western Ontario) A London, Canada, scientist studying cystic fibrosis has successfully corrected the defect which causes the overproduction of intestinal mucous in mice.

This discovery by Dr. Richard Rozmahel, a scientist with the Lawson Health Research Institute, affiliated with the University of Western Ontario, has clear implications to understanding and treating this facet of the disease in humans.

CF is a fatal, genetic disease characterized by an overproduction of mucous in the lungs and digestive system.

Dry airways may not only play a central role in the development of the inherited lung disease cystic fibrosis, but also in acquired chronic lung diseases like asthma and smoker's lung, the cigarette smoke-induced chronic obstructive pulmonary disease (COPD).

Researchers found, in animal studies, that insufficient hydration of the airways leads to pathologies typical of COPD in humans.