TABLE 1.

Molecules that interact with CFTRa

MoleculeFunctionInteraction with CFTRReference(s)
Protein channels
    ORCCOutwardly rectifying chloride channelCFTR required for activation by PKA and PKC140, 141, 284, 286
    ENaCEpithelial sodium channelNBD-1, R domain binding inhibits ENaC function166, 273
    ROMK2Renally derived K+-ATP channelTMD1, NBF1, and R domain of CFTR interact46
    Aquaporin 3     (AQP3)Water channels in airway epithelial cellsCFTR-dependent activation via NBD1274, 275
Transporters
    NKCC1Na+/K+/2Cl cotransporterWild-type CFTR upregulates expression296
    NBC-1Electrogenic Na+/HCO3 cotransporterCFTR-Cl secretion potentiates NBC function295
Other proteins
    Syntaxin 1AMembrane traffic machineryBinds 1:1 CFTR N terminus, inhibits CFTR function202, 203
    NHERF/EBP50Na+/H+ exchanger regulatory factor/ERM- binding phosphoprotein 50Binds to PDZ motif at CFTR C terminus31, 119, 294
  • a Reprinted from reference 229a with permission from Elsevier Science.