Mutations

ID #2256

In one of my notes for cystic fibrosis, it says "causes Cl- to accumulate in cells, causing water to be taken into cells." What exactly does Cl- do?


The CFTR protein in epithelial cells helps to produce mucus. Mucus helps cleanse and lubricate many passageways in the body, including the airway in the lungs. The role of the CFTR protein is to allow chloride ions to exit the mucus-producing cells. When the chloride ions leave these cells, water follows, thinning the mucus. In this way, the CFTR protein helps to keep mucus from becoming thick.
In CF patients, the defective CFTR protein does not allow chloride ions out of mucus-producing cells. With less chloride leaving the cells, less water leaves, and mucus becomes thick and sticky. The mucus can no longer move freely through the passageways, and they become clogged.

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