Cystic fibrosis can be deadly Researchers have discovered a gene
that modifies the severity of lung disease in people with the
lethal genetic condition cystic fibrosis. The US study,
published by Nature, may aid development of new treatments. The
researchers deleted the key gene in mice, and then exposed the
animals to a bacterium which often causes lung infections in
cystic fibrosis patients. The animals showed less sign of
inflammation and disease than would otherwise be expected.
Around 8,000 people in the UK have cystic fibrosis and one in 25
are carriers for the condition. Cystic fibrosis produces thick,
sticky mucus that clogs the lungs and the pancreas, leading to
life-threatening chest infections and problems with digestion.
Existing treatments only ease symptoms and life expectancy is
low - most with people with cystic fibrosis die before they
reach 40. It has been known for 20 years that cystic fibrosis is
caused by mutations in a gene called CFTR. However, it has
become apparent in recent years tha