Human Cystic Fibrosis Transmembrane Conductance Regulator, CFTR ELI

Epithelial ion Channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis (PubMed: 26823428).

Mediates the transport of chloride ions across the cell membrane (PubMed: 10792060, PubMed: 11524016, PubMed: 11707463, PubMed: 12519745, PubMed: 15010471, PubMed: 12588899, PubMed: 17036051, PubMed: 19398555, PubMed: 19621064, PubMed: 22178883, PubMed: 25330774, PubMed: 1712898, PubMed: 8910473, PubMed: 9804160, PubMed: 12529365, PubMed: 17182731, PubMed: 26846474, PubMed: 28087700).

Channel activity is coupled to ATP hydrolysis (PubMed: 8910473).

The ion Channel is also permeable to HCO(3-); selectivity depends on the extracellular chloride concentration (PubMed: 15010471, PubMed: 19019741).

Exerts its function also by modulating the activity of other ion Channels and transporters (PubMed: 12403779, PubMed: 22178883, PubMed: 22121115, PubMed: 27941075).

Plays an important role in airway fluid homeostasis (PubMed: 16645176, PubMed: 19621064, PubMed: 26823428).

Contributes to the regulation of the pH and the ion content of the airway surface fluid layer and thereby plays an important role in defense against pathogens (PubMed: 14668433, PubMed: 16645176, PubMed: 26823428).

Modulates the activity of the epithelial sodium Channel (ENaC) complex, in part by regulating the cell surface expression of the ENaC complex (PubMed: 17434346, PubMed: 27941075, PubMed: 17182731).

Inhibits the activity of the ENaC Channel containing subunits SCNN1A, SCNN1B and SCNN1G (PubMed: 17182731).

Inhibits the activity of the ENaC Channel containing subunits SCNN1D, SCNN1B and SCNN1G, but not of the ENaC Channel containing subunits SCNN1A, SCNN1B and SCNN1G (PubMed: 27941075).

May regulate bicarbonate secretion and salvage in epithelial cells by regulating the transporter SLC4A7 (PubMed: 12403779).

Can inhibit the chloride Channel activity of ANO1 (PubMed: 22178883).

Plays a role in the chloride and bicarbonate homeostasis during sperm epididymal maturation and capacitation (PubMed: 19923167, PubMed: 27714810).