Normal CFTR protein
CFTR proteins help maintain fluid balance
The cystic fibrosis transmembrane conductance regulator (CFTR) gene tells our cells how to make CFTR proteins.
Once CFTR proteins have been processed and folded correctly inside the cell, they are transported to the cell surface (membrane).
CFTR proteins are found at the surface of cells in many parts of the body, including the lungs, sweat glands, intestines, pancreas, sinuses, and reproductive system.
CFTR proteins in a normal cell
Salt, water, and mucus
By controlling the flow of ions in and out of cells, CFTR proteins help make sure that there is the right balance of salt and water in our organs. The correct balance of salt and water is needed to help keep mucus, a slippery substance produced naturally by the body, thin and watery. Mucus covers and protects the lining of our airways, digestive system, reproductive system, and other organs and tissues in the body.
Here is an example of how mucus works to protect organs in the body:
- The cells of your airway surfaces are covered in fine hair-like projections called cilia
- Cilia are constantly working to brush away thin, watery mucus containing trapped dirt and germs
- This process sweeps mucus out towards the windpipe, where it is either coughed out or swallowed
So you see, it’s important that mucus stays thin and watery so that your organs can function normally.
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Wang W, Linsdell P. Conformational change opening the CFTR chloride channel pore coupled to ATP-dependent gating. Biochim Biophys Acta. 2012; 1818(3): 851–860.