Protein opens hope of treatment for cystic fibrosis patientsSeptember 11, 2008Scientists have finally identified a direct role for the missing protein that leaves cystic fibrosis patients open to attack from lung-damaging bacteria, the main reason most of them die before their 35th birthday, scientists heard today (Thursday 11 September 2008) at the Society for General Microbiology's Autumn meeting being held this week at Trinity College, Dublin. "Chronic lung infections are by far and away the biggest problem with the genetic disease cystic fibrosis, affecting almost 90% of patients. The majority of these bad infections are caused by one particular type of bacteria called Pseudomonas aeruginosa," said Professor Gerald Pier from Harvard Medical School in Boston, USA. "Once a chronic infection starts in a cystic fibrosis patient it is almost never cleared and will go on getting worse and causing lung damage over many years before killing the patient by the age of 35 years." The US scientists trying to work out why cystic fibrosis patients get this infection have discovered that the protein called CFTR that is either missing or not working properly in their lungs is needed by our bodies to recognise when Pseudomonas aeruginosa bacteria are inhaled. People whose CFTR protein is working correctly can rapidly clear the infectious bacteria out of their lungs.
"In cystic fibrosis patients the recognition system is deficient or absent, and the patient does not rapidly expel the bacteria, allowing the microbes to settle into the lungs and cause a chronic infection," said Professor Pier. By understanding how our lung cells use CFTR to recognize and properly respond to Pseudomonas aeruginosa infections the Harvard team hopes that they will be able to identify points of intervention that could be activated in cystic fibrosis lungs to increase their resistance to infection. "At the moment our research is more geared to identifying the molecules that lung cells use to resist infection, although our longer term goal is to find ways to restore the resistance of the lung to infection," said Professor Pier. "Our major path right now is to further understand the general process of resistance to infection, using cystic fibrosis as a model as well as a real disease. But the next step will be to try some drug interventions to improve the resistance of cystic fibrosis patients to infection which will undoubtedly prolong their lives and enhance the quality of their life as well". Society for General Microbiology | |||||||||||||||||||||
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Related Cystic Fibrosis Current Events and Cystic Fibrosis News Articles Researchers describe how cells take out the trash to prevent disease Garbage collectors are important for removing trash; without them waste accumulates and can quickly become a health hazard. Similarly, individual cells that make up such biological organisms as humans also have sophisticated methods for managing waste. Children with cystic fibrosis not well covered by guidelines for vitamin D needs Existing recommendations for treating vitamin D deficiency in children with cystic fibrosis (CF) are too low to cover the serious need, leaving most at high risk for bone loss and rickets, according to researchers at Johns Hopkins Children's Center. New approach to treating cystic fibrosis lung infection shows promise Researchers at the University of Calgary have found a new method of fighting severe lung infections in people with cystic fibrosis (CF). These findings are published in Proceedings of the National Academy of Science USA, this week. Rattlesnake-type poisons used by superbug bacteria to beat our defenses Colonies of hospital superbugs can make poisons similar to those found in rattlesnake venom to attack our bodies' natural defences, scientists heard today (Monday 8 September 2008) at the Society for General Microbiology's Autumn meeting being held this week at Trinity College, Dublin. Clearing the airways in cystic fibrosis 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. PTC124 shows activity in cystic fibrosis; Phase 2 proof-of-concept data published in Lancet New phase 2 data published today in The Lancet show that the investigational oral drug PTC124 demonstrates activity in nonsense-mutation cystic fibrosis (CF). The data show that treatment with PTC124 results in statistically significant improvements in the chloride channel function of patients with nonsense-mutation CF. The study was conducted at the Hadassah Hebrew University Hospital in Jerusalem, Israel and sponsored by PTC Therapeutics (PTC). Breaking the 'mucus barrier' with a new drug delivery system Chemical engineers from Johns Hopkins University 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 noted in a presentation scheduled for the 236th National Meeting of the American Chemical Society. NIH scientists find a novel mechanism that controls the development of autoimmunity Scientists at the National Institutes of Health (NIH) have found a mechanism in the immune systems of mice that can lead to the development of autoimmune disease when turned off. Researchers discover cell's 'quality control' mechanism 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 (CF) and some other hereditary diseases, the researchers say. Their results were published July 25 in the journal Science. Most generalists reluctant to provide primary care for young adults with chronic illness The majority of general internists and pediatricians in the United States are not comfortable serving as primary care providers for young adults with complex chronic illnesses that originate during childhood, according to findings from a new national survey. More Cystic Fibrosis Current Events and Cystic Fibrosis News Articles |
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