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Latest Cystic fibrosis transmembrane conductance regulator Stories

2013-09-11 23:20:17

ChanTest is a world leader in drug safety and discovery, in areas particularly relevant to Cystic Fibrosis. Cleveland, OH (PRWEB) September 11, 2013 The world is still looking for meaningful solutions to address the pain and suffering of patients with Cystic Fibrosis. On Thursday, September 12, ChanTest will be hosting a complimentary webinar entitled “Unfolding Cystic Fibrosis Research,” to share important information in this field. The event will include presentations by Dr....

2013-03-22 09:42:47

Data about defective regulatory protein described in Proceedings of the National Academy of Sciences A little more than a year after the FDA approved Kalydeco (Vx-770), the first drug of its kind to treat the underlying cause of cystic fibrosis, University of Missouri researchers believe they have found exactly how this drug works and how to improve its effectiveness in the future. Described in the current issue of the Proceedings of the National Academy of Sciences, MU researchers have...

2012-03-12 14:31:32

New research could provide roadmap for more effective drug discovery for CF A recent study led by Gergely Lukacs, a professor at McGill University's Faculty of Medicine, Department of Physiology, and published in the January issue of Cell, has shown that restoring normal function to the mutant gene product responsible for cystic fibrosis (CF) requires correcting two distinct structural defects. This finding could point to more effective therapeutic strategies for CF in the future. CF, a...

2011-09-19 21:57:12

Cystic fibrosis (CF), a chronic disease that clogs the lungs and leads to life-threatening lung infections, is caused by a genetic defect in a chloride channel called cystic fibrosis transmembrane conductase regulator (CFTR). Although scientists do not fully understand how or why this defect occurs, a team of researchers at The Hospital for Sick Children (SickKids) in Toronto, Ontario, Canada has found a promising clue: a protein called ubiquitin ligase Nedd4L. In a study led by Daniela...

2011-09-19 21:56:15

When researchers discovered the primary genetic defect that causes cystic fibrosis (CF) back in 1989, they opened up a new realm of research into treatment and a cure for the disease. Since then, scientists have been able to clone the defective gene and study its effects in animals. Now researchers at the University of North Carolina at Chapel Hill have developed a technique for observing the defects at work in human tissue donated by patients with CF. This technique has yielded an...

2011-09-01 16:42:25

Researchers have identified an unconventional path that may correct the defect underlying cystic fibrosis, according to a report in the September 2nd issue of the journal Cell, a Cell Press publication. This new treatment dramatically extends the lives of mice carrying the disease-associated mutation. Cystic Fibrosis is caused by a mutation in a gene responsible for the transport of ions across cell membranes. This gene encodes a protein channel, called the cystic fibrosis transmembrane...

2010-11-15 12:12:44

Researchers at the University of North Carolina at Chapel Hill School of Medicine have demonstrated that the gene mutated in cystic fibrosis not only controls traffic on the chloride highway, but also keeps the sodium highway from being overused. An imbalance of salt and water in patients with cystic fibrosis makes their lungs clog up with sticky mucus that is prone to infection. The cause of the offending imbalance is a well-known genetic error, one that blocks the molecular expressway for...

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2010-09-20 07:52:26

Crosstalk between ion channels points to new therapeutic strategy, Penn study finds The lab of Kevin Foskett, PhD, the Isaac Ott Professor of Physiology at the University of Pennsylvania School of Medicine, has found a possible new target for fighting cystic fibrosis (CF) that could compensate for the lack of a functioning ion channel in affected CF-related cells. Their finding appears in the Journal of Clinical Investigation. The team explored the role of CFTR, the chloride ion channel...

2009-12-18 13:55:39

Investigators report new findings about its special relationship with pH levels inside cells A team of researchers studying the protein that, when defective or absent, causes cystic fibrosis (CF) has made an important discovery about how that protein is normally controlled and under what circumstances it might go awry. "Understanding the regulation of salt transport in normal cells is critical for the development of new therapies for diseases, like CF, that disrupt salt movements across cell...

2009-09-09 09:02:13

Researchers at the University of North Carolina at Chapel Hill have discovered a genetic risk factor for severe liver disease in people with cystic fibrosis. Those who carry a particular variant of the SERPINA1 gene (also known as alpha-1-antitrypsin or alpha-1-antiprotease) are five times more likely to develop cirrhosis and other liver complications than patients who carry the normal version of the gene. The study, which appears in the Sept. 9 issue of the Journal of the American Medical...


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