“The normal function of a cell is to pass chloride ions across the cell membrane at a very fast speed,” Hwang said. “We know some signaling molecules elicit this reaction, much like a hand signals an automatic water faucet to dispense water. But in the case of cystic fibrosis, that signal is no longer detected by the mutated channel protein. Through some mechanisms we still don’t quite understand, malfunction of this channel protein eventually leads to bacterial infection in the lung, which is believed to be responsible for the most severe symptoms of cystic fibrosis.”Read the whole story using the link below
The most recent study found that manipulating the sensor of the channel protein can significantly rectify the malfunction of the mutated channel, thus opening the door to a drug design that may eventually be a “real cure,” Hwang said.