Tough mucus, settle in the bacteria that cause then chronic infection forms Munich (netdoktor.de) – case of cystic fibrosis in the Airways. A recent study comes to the conclusion that the previous opinion of textbook, such as this mucus is produced, is wrong. That could have any practical effect on the therapy. The researchers report the Westfälische Wilhelms Universität Münster (EMU) in the journal “Pathobiology” (DOI: 10.1159 / 000295859).

The cause of the incurable disorder cystic fibrosis is clear: “mutations on the long arm of chromosome 7″ prevent, that a particular protein is properly formed, explains Dr. Tobias Schulz of the EMU. The protein is the so-called (CFTR) chloride channel: he sits in the cell membrane and transports chloride ions to the outside. Osmotic reasons follows passive water in the same direction and ensures a liquefaction of bronchial secretions in the respiratory tract. Due to the mutation-related channel this salt and therefore water transport would be disturbed, making the mucus become viscous – so the previous doctrine.

Schulz and colleagues have now discovered that chloride channels have a second function that plays a role for the formation of mucus. “We could find that the chloride channels can be passed by the hyaluronic acid”, so Schulz. “This is surprising.” “Actually, these molecules is among the largest in the human body – and bind much more water than chloride ions.” The hyaluronic acid-export does not work in cystic fibrosis patients. Apparently, this is the main cause for the emergence of tough mucus.

The new findings are of practical importance. On its basis, the scientists have developed a new substance that could significantly improve the treatment of cystic fibrosis patients: the starting point is the fact that the channel proteins in spite of the mutant gene are formed and are also functional. Because they but differ slightly in their structure from the “original”, they are mined in the cell before it can begin its work in the cell membrane. The new substance prevents the degradation of proteins. It is patent pending. (mf)