The CFTR receptor (an ion channel, which transports CL- out of the cell) can form a complex with CFTR assiciated protein (CAL), followed by degradation. If NHERF1 is present it will form a complex with the CFTR receptor (by binding the PDZ1 region), inhibiting degradation. This complex will bind to other proteins / ion channels. One of these ion channels is the Slc26 family, which binds to the PDZ2 region of NHERF1. After forming this complex Cl- and NaHCO3 secretion will be performed. The same thing happens with the ENaC (an ion channel, which transports Na+), which consists of 3 subunits (alfa, beta & gamma). This channels binds YES-accosiated protein (YAP), which in his turn binds the PDZ2 region of NHERF1. Also c-YES will bind to NHERF1, which inhibits the ENaC channel. By forming this complex Cl- secretion will be induced, while Na+ absorption will be reduced.

Authors

Flipke , Thomas Kelder , Kristina Hanspers , Daniela Digles , Egon Willighagen , and Martina Summer-Kutmon

Cited In

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Organism

Rattus norvegicus

Communities

Annotations

Pathway Ontology: chloride ion transport pathway

Participants

References

1. New insights into cystic fibrosis: molecular switches that regulate CFTR. Guggino WB, Stanton BA. Nat Rev Mol Cell Biol. 2006 Jun;7(6):426–36. PubMed Europe PMC Scholia