Normal CFTR protein
CFTR proteins help maintain fluid balance
Once the CFTR proteins have been made they are sent to the cell surface. Here, they act like channels with gates, which open and close to allow water and particles such as
This balance of salt and water is needed to keep the mucus protecting our airways and digestive tract thin and watery. This ensures organs such as the lungs,
CFTR proteins in a normal cell
*CFTR = Cystic fibrosis transmembrane conductance regulator
Wang W, Linsdell P. Conformational change opening the CFTR chloride channel pore coupled to ATP-dependent gating. Biochimica et Biophysica Acta (BBA) - Biomembranes 2012; 1818(3): 851 ̶ 860.
Macdonald KD, et al. Cystic Fibrosis Transmembrane Regulator Protein Mutations. Pediatr Drugs 2007; 9(1): 1 ̶ 10.