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CF is caused by mutations in the cystic fibrosis transmembrane conductance regulator ( CFTR) gene, which result in a defect and/or reduction in the amount of CFTR protein that the body makes. Children must inherit two defective CFTR genes — one from each parent — to have CF. The CFTR protein is known to acts as a chloride (Cl-) channel expressed in the exocrine glands of several body systems where it also regulates other ion channels, including the epithelial sodium (Na +) channel (ENaC) that plays a key role in salt absorption.
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