A new discovery by Californian scientists may lead to a pharmaceutical breakthrough for a wide range of illnesses that involve the hydration of cells that line the inner surfaces of our body's organs and tissues. In a new report appearing in the FASEB Journal *, the researchers describe how they used high-throughput screening to identify small-molecule drug candidates which help cells bypass defective channels that normally move salt and water through cell membranes. These drug candidates work by activating an alternative chloride channel called "TMEM16A" that might be effective in treating conditions such as cystic fibrosis, dry eye and dry mouth diseases and slow-transit constipation. "Further pre-clinical development of the chloride channel activators identified in our study may lead to new drug therapies for cystic fibrosis, dry eye and mouth syndromes, and certain types of constipation, " said Alan S.
New research in The FASEB Journal suggests that cigarette smoke interferes with the Cystic Fibrosis Transmembrane Conductance Regulator protein, leading to dry, sticky mucus and increased infections. If you smoke cigarettes, you have more in common with someone who has cystic fibrosis than you think. A new research report appearing online in the FASEB Journal shows that smoking cigarettes affects the lungs in a way that is very similar to cystic fibrosis, a life threatening disease affecting the lungs and other organs. In cystic fibrosis, improper movement of salt and water in the cells lining the lungs causes a thick and sticky mucus to form. Bacteria become trapped in this mucus and thrive, leading to life-threatening lung infections. The FASEB Journal study shows that smoking negatively affects the lungs in a similar way, leading to mucus that causes dry cough, chronic bronchitis, and chronic obstructive pulmonary disease, among other problems.
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 extraordinary view of the cellular intricacies of CF, which Martina Gentzsch, assistant professor of cell and developmental biology, discussed at the 7th International Symposium on Aldosterone and the ENaC/Degenerin Family of Ion Channels, being held September 18-22 in Pacific Grove, Calif. The meeting is sponsored by the American Physiological Society.
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 Rotin, PhD, senior scientist at SickKids and professor of biochemistry at the University of Toronto, mice specially bred to lack Nedd4L in the lung developed cystic fibrosis-like lung disease. Dr. Rotin, the lead author of a number of recently published studies on this topic, will discuss the team's findings at the 7th International Symposium on Aldosterone and the ENaC/Degenerin Family of Ion Channels conference sponsored by the American Physiological Society.
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 conductance regulator or CFTR, that is normally found on the surfaces of cells lining the airway and intestine. In patients with the disease, the channels don't make it from inside cells to their surfaces along the standard path. As a result ions and fluids fail to move in and out of cells as they should, causing mucus build-up and chronic lung infections.