What are the Systemic implications of defects in the CFTR channel ?

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What are the Systemic implications of defects in the CFTRchannel ?

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CFTR cystic fibrosis transmembrane conductance regulator is the protein that forms CFTR channels in apical membrane of epithelial cells in organs such as lungs and pancreas The CFTR channels are involved in transport of chloride from cytoplasm of epithelial cells to the extracellular medium Chloride ions accumulate within epithelial cells due to actions of anion exchanged and NBC present in basolateral membrane of the epithelial cells CFTR transport of Cl ions will cause sodium ions to also move out of the cell Sodium ions move along with water via a paracellular pathway from blood into the epithelial lumen Water can also move into extracellular lumen via aquaporin channels The sodium chloride and water will cause mucus to be diluted in extracellular lumen Although CFTR can also transport bicarbonate ions its major role is to transport See Answer

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