Cystic

Scientists discover basic defect in cystic fibrosis airway glands

Scientists at Stanford University have determined that the buildup of sticky mucus found in cystic fibrosis is caused by a loss in the epithelial cell's ability to secrete fluid. This research appears as the "Paper of the Week" in the March 17 issue of the Journal of Biological Chemistry, an American Society for Biochemistry and Molecular Biology journal. Cystic fibrosis is the most commo...

Study reveals how cells destroy faulty proteins in cystic fibrosis

The cellular system that degrades faulty proteins created by the cystic fibrosis gene has been identified by University of North Carolina at Chapel Hill scientists. Turning off the degradation system allows some proteins to regain their proper shape, offering a new avenue for treatments aimed at curing the disease. Cystic fibrosis (CF) is a fatal disease caused by a defective gene that...

Discovery could aid fight against cystic fibrosis infection

Harvard Medical School researchers have discovered one way that a hardy disease-causing bacteria could be surviving in the lungs of chronically infected cystic fibrosis (CF) patients. "This work is important because pathogenic bacteria such as Pseudomonas aeruginosa (PA) use protein secretion systems to cause disease in their hosts," said Joseph Mougous, lead author of the study publishe...

Clues to gene expression in cystic fibrosis will guide research

Genetics tests could help provide cystic fibrosis (CF) patients with targeted treatment in future, pilot study authors suggest. Results from a French clinical trial published today in BMC Medicine show how a small percentage of CF sufferers with a rare genetic stop mutation responded positively to gentamicin treatment. Aleksander Edelman and Isabelle Sermet-Gaudelus of Faculté de Médicine...

Key found to kill cystic fibrosis superbug

Researchers from the Schulich School of Medicine & Dentistry at The University of Western Ontario , working with a group from Edinburgh, have discovered a way to kill the cystic fibrosis superbug, Burkholderia cenocepacia. These investigators, under the leadership of Dr. Miguel Valvano, Department of Microbiology and Immunology, have had their research published in the May issue of the Journa...

Penn study on lung-infecting bacterial enzyme suggests new approach to cystic fibrosis treatment

Researchers at the University of Pennsylvania School of Medicine have discovered that an enzyme produced by lung-infecting bacteria further shuts down a protein that is defective in cystic fibrosis patients. The disruption to this protein that conveys ions from lung cells to airways causes thick mucus to buildup inside the lung. The finding suggests a new therapeutic target for treating lung infe...