Summary

Name CFTR
Full Name Cystic fibrosis transmembrane conductance regulator
Synonyms CFTR, ATP-binding cassette sub-family C member 7, Channel conductance-controlling ATPase, 3.6.3.49, cAMP-dependent chloride channel | ABCC7
Primary ID P13569
Links - -
Type protein
Relations 35
Inhibitors ivacaftor; glyburide; tolbutamide
Function 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).

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Modifications Tables

Relations

Regulator Mechanism target score
+ up-regulates img/direct-activation.png phosphorylation CFTR 0.281
Publications: 1 Organism: Homo Sapiens
+ down-regulates img/direct_inhibition.png phosphorylation CFTR 0.281
Publications: 2 Organism: Homo Sapiens
+ up-regulates img/direct-activation.png phosphorylation CFTR 0.521
Publications: 6 Organism: Homo Sapiens
+ up-regulates activity img/direct-activation.png phosphorylation CFTR 0.466
Publications: 3 Organism: In Vitro
+ up-regulates img/direct-activation.png phosphorylation CFTR 0.466
Publications: 3 Organism: Homo Sapiens
+ up-regulates activity img/direct-activation.png phosphorylation CFTR 0.408
Publications: 2 Organism: In Vitro
+ up-regulates activity img/direct-activation.png phosphorylation CFTR 0.521
Publications: 1 Organism: In Vitro
+ down-regulates activity img/direct_inhibition.png phosphorylation CFTR 0.474
Publications: 2 Organism: Homo Sapiens
+ down-regulates activity img/direct_inhibition.png phosphorylation CFTR 0.466
Publications: 2 Organism: Homo Sapiens
+ down-regulates activity img/direct_inhibition.png phosphorylation CFTR 0.396
Publications: 2 Organism: Homo Sapiens
+ down-regulates activity img/direct_inhibition.png phosphorylation CFTR 0.353
Publications: 2 Organism: Homo Sapiens, Mus Musculus
Tissue: Pancreas
+ down-regulates quantity img/direct_inhibition.png binding CFTR 0.658
Publications: 1 Organism: Homo Sapiens
+ up-regulates activity img/direct-activation.png dephosphorylation CFTR 0.2
Publications: 1 Organism: Mus Musculus
Tissue: Pancreas
+ down-regulates img/direct_inhibition.png chemical inhibition CFTR 0.8
Publications: 1 Organism: Homo Sapiens
+ down-regulates activity img/direct_inhibition.png chemical inhibition CFTR 0.8
Publications: 1 Organism: Mus Musculus
+ down-regulates activity img/direct_inhibition.png chemical inhibition CFTR 0.8
Publications: 1 Organism: Mus Musculus
+ down-regulates quantity by destabilization img/direct_inhibition.png polyubiquitination CFTR 0.484
Publications: 1 Organism: Homo Sapiens
+ up-regulates quantity by expression img/indirect-activation.png transcriptional regulation CFTR 0.256
Publications: 1 Organism: Homo Sapiens
+ down-regulates img/direct_inhibition.png binding CFTR 0.711
Publications: 1 Organism: Homo Sapiens
+ down-regulates quantity by destabilization img/direct_inhibition.png ubiquitination CFTR 0.651
Publications: 1 Organism: Homo Sapiens