Cpr Structure
CFTR is a plasma membrane protein that regulates the level of chloride and sodium in normal cells. When there is a mutation in the CFTR gene, it is not able to function properly leading to a build-up of abnormally thick mucus, caused by the dead inflammatory cells increasing its viscosity, in the body’s passages.
CFTR acts as a chloride channel, pumping chlorine ions out of the cell and is responsible for regulating the other channels. The protein consists of three motifs;
- TM1 an TM2;
- NBF1 and NBF2
- Unique structure of 230 amino acids.
When the NBF1 region is active, it acts as an inhibitor for epithelial Sodium Channel. When CFTR is inactive the balance of chlorine within a cell shifts as the epithelial sodium channel is no longer
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