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CFTR Gene Record

  • Summary
  • Interactions
  • Claims
  • CFTR 1080 Druggable Genome

    Alternate Names:

    1080
    CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR
    CFTR
    ABC35
    ABCC7
    CF
    CFTR/MRP
    MRP7
    TNR-CFTR
    dJ760C5.1
    602421
    1884
    ENSG00000001626
    OTTHUMG00000023076
    707
    P13569
    cAMP-dependent chloride channel
    Channel conductance-controlling ATPase
    ATP-binding cassette sub-family C member 7
    PA109
    CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR (CFTR) (CAMP- DEPENDENT CHLORIDE CHANNEL). [SOURCE:UNIPROT/SWISSPROT;ACC:P13569]
    BE0001195
    CFTR_HUMAN
    T55654
    CF transmembrane conductance regulator

    Gene Info:

    GuideToPharmacology Gene Category Name CFTR
    GuideToPharmacology Gene Category ID 129
    Target Class Transporters
    Target Subclass TC:3.A.1.202.1
    Human Readable Name DRUGGABLE GENOME
    Interpro Type Domain
    Uniprot Evidence 1: Evidence at protein level
    Human Readable Name ABC TRANSPORTER
    Interpro Acc IPR001140
    Uniprot Status Swiss-Prot
    Interpro Short Name ABC_transptr_TM_dom
    Interpro Name ABC transporter, transmembrane domain
    Gene Biotype PROTEIN_CODING
    (12 More Sources)

    Gene Categories: Category Details

    TRANSPORTER
    CELL SURFACE
    ION CHANNEL
    ABC TRANSPORTER
    DRUGGABLE GENOME

    Publications:

    Pesce E et al., 2015, Synthesis and structure-activity relationship of aminoarylthiazole derivatives as correctors of the chloride transport defect in cystic fibrosis., Eur J Med Chem
    Lubamba B et al., 2012, Cystic fibrosis: insight into CFTR pathophysiology and pharmacotherapy., Clin Biochem
    Ikpa PT et al., 2014, Cystic fibrosis: toward personalized therapies., Int J Biochem Cell Biol
    Awatade NT et al., 2014, Measurements of Functional Responses in Human Primary Lung Cells as a Basis for Personalized Therapy for Cystic Fibrosis., EBioMedicine
    Compain P et al., 2013, Rescue of functional CFTR channels in cystic fibrosis: a dramatic multivalent effect using iminosugar cluster-based correctors., Chembiochem
    Yu et al., 2012, Ivacaftor potentiation of multiple CFTR channels with gating mutations., J. Cyst. Fibros.
    Kuk et al., 2015, Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects., Ther Adv Respir Dis
    Boyle MP et al., 2014, A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial., Lancet Respir Med
    Clancy JP et al., 2012, Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation., Thorax
    Norman P, 2014, Novel picolinamide-based cystic fibrosis transmembrane regulator modulators: evaluation of WO2013038373, WO2013038376, WO2013038381, WO2013038386 and WO2013038390., Expert Opin Ther Pat
    Veit G et al., 2016, From CFTR biology toward combinatorial pharmacotherapy: expanded classification of cystic fibrosis mutations., Mol Biol Cell
    Hubert D et al., 2017, Real-life initiation of lumacaftor/ivacaftor combination in adults with cystic fibrosis homozygous for the Phe508del CFTR mutation and severe lung disease., J Cyst Fibros
    Kopeikin Z et al., 2014, Combined effects of VX-770 and VX-809 on several functional abnormalities of F508del-CFTR channels., J Cyst Fibros
    Dekkers JF et al., 2016, Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis., Sci Transl Med
    Van Goor F et al., 2011, Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809., Proc Natl Acad Sci U S A
    Van Goor F et al., 2014, Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function., J Cyst Fibros
    Fohner AE et al., 2017, PharmGKB summary: ivacaftor pathway, pharmacokinetics/pharmacodynamics., Pharmacogenet Genomics
    Wang W et al., 2016, Robust Stimulation of W1282X-CFTR Channel Activity by a Combination of Allosteric Modulators., PLoS One
    Tradtrantip et al., 2010, Crofelemer, an antisecretory antidiarrheal proanthocyanidin oligomer extracted from Croton lechleri, targets two distinct intestinal chloride channels., Mol. Pharmacol.
    Arora K et al., 2016, Personalized medicine in cystic fibrosis: genistein supplementation as a treatment option for patients with a rare S1045Y-CFTR mutation., Am J Physiol Lung Cell Mol Physiol
    Derand et al., 2002, The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory Genistein binding site., J. Biol. Chem.
    Devor et al., 1998, Ibuprofen inhibits cystic fibrosis transmembrane conductance regulator-mediated Cl- secretion., J. Clin. Invest.
    Kerem E et al., 2008, Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial., Lancet
    Wilschanski M et al., 2011, Chronic ataluren (PTC124) treatment of nonsense mutation cystic fibrosis., Eur Respir J
    Shoseyov D et al., 2016, Ataluren for the treatment of cystic fibrosis., Expert Rev Respir Med
    Sermet-Gaudelus I et al., 2010, Ataluren (PTC124) induces cystic fibrosis transmembrane conductance regulator protein expression and activity in children with nonsense mutation cystic fibrosis., Am J Respir Crit Care Med
    Tosco A et al., 2016, A novel treatment of cystic fibrosis acting on-target: cysteamine plus epigallocatechin gallate for the autophagy-dependent rescue of class II-mutated CFTR., Cell Death Differ
    Donaldson SH et al., 2018, Tezacaftor/Ivacaftor in Subjects with Cystic Fibrosis and F508del/F508del-CFTR or F508del/G551D-CFTR., Am J Respir Crit Care Med
    Saint-Criq V et al., 2017, Role of CFTR in epithelial physiology., Cell Mol Life Sci
    Rowe SM et al., 2017, Tezacaftor-Ivacaftor in Residual-Function Heterozygotes with Cystic Fibrosis., N Engl J Med
    Taylor-Cousar JL et al., 2017, Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del., N Engl J Med
    Reddy et al., 1999, Bumetanide blocks CFTR GCl in the native sweat duct., Am. J. Physiol.
    Hutt DM et al., 2010, Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis., Nat Chem Biol
    Zhou et al., 2002, Probing an open CFTR pore with organic anion blockers., J. Gen. Physiol.
    Reddy et al., 2002, Effect of anion transport blockers on CFTR in the human sweat duct., J. Membr. Biol.
    Jiang et al., 2003, Pleural surface fluorescence measurement of Na+ and Cl- transport across the air space-capillary barrier., J. Appl. Physiol.
    Larsen et al., 2003, Beta-adrenergic receptors couple to CFTR chloride channels of intercalated mitochondria-rich cells in the heterocellular toad skin epithelium., Biochim. Biophys. Acta
    Lee et al., 2005, Inhibition of Na+-K+ pump and L-type Ca2+ channel by glibenclamide in Guinea pig ventricular myocytes., J. Pharmacol. Exp. Ther.
    Berger AL et al., 2005, Normal gating of CFTR requires ATP binding to both nucleotide-binding domains and hydrolysis at the second nucleotide-binding domain., Proc Natl Acad Sci U S A
    Ramesh Babu PB et al., 2004, Regulation of apical surface fluid and protein secretion in human airway epithelial cell line Calu-3., Biochem Biophys Res Commun
    Berman et al., 2000, The Protein Data Bank., Nucleic Acids Res.
    Sommer D et al., 2007, CFTR-dependent Cl- secretion in Xenopus laevis lung epithelium., Respir Physiol Neurobiol
  • LUMACAFTOR   CFTR

    Interaction Score: 45.44

    Interaction Types & Directionality:
    modulator

    Interaction Info:
    Trial Name VX-809
    Novel drug target Established target
    Trial Name VX-809 + VX-770

    PMIDs:
    26041577 22698459 24561283 26137539 24038832 22293084 26416827 24973281 21825083 24392786 26823392 28325531 24796242 27334259 21976485 23891399


    Sources:
    DTC FDA PharmGKB TdgClinicalTrial TTD DrugBank GuideToPharmacology ChemblInteractions

  • TEZACAFTOR   CFTR

    Interaction Score: 18.94

    Interaction Types & Directionality:
    activator (activating)

    Interaction Info:
    Mechanism of Interaction Cystic fibrosis transmembrane conductance regulator positive modulator
    Direct Interaction yes
    Details of the Assay for Interaction Measuring the increase in cAMP/genistein-dependent current amplitude via the ΔF508-CFTR mutant expressed in NIH/3T3 cells by patch-clamp recording, in response to tezacaftor exposure.

    PMIDs:
    28930490 27714410 29099333 29099344


    Sources:
    FDA PharmGKB TTD DrugBank GuideToPharmacology ChemblInteractions

  • IVACAFTOR   CFTR

    Interaction Score: 7.57

    Interaction Types & Directionality:
    activator (activating)
    potentiator

    Interaction Info:
    Trial Name VX-809 + VX-770
    Novel drug target Established target
    Trial Name VX-770

    PMIDs:
    27636560 22293084


    Sources:
    FDA TdgClinicalTrial TTD DrugBank GuideToPharmacology ChemblInteractions

  • ELEXACAFTOR   CFTR

    Interaction Score: 5.68

    Interaction Types & Directionality:
    modulator

    Interaction Info:
    Specific Action of the Ligand Binding
    Endogenous Drug? False
    Direct Interaction? False

    PMIDs:
    None found


    Sources:
    FDA DrugBank GuideToPharmacology

  • CROFELEMER   CFTR

    Interaction Score: 5.68

    Interaction Types & Directionality:
    antagonist (inhibitory)
    inhibitor (inhibitory)

    Interaction Info:
    Mechanism of Interaction Cystic fibrosis transmembrane conductance regulator inhibitor
    Direct Interaction yes
    Trial Name crofelemer

    PMIDs:
    19808995


    Sources:
    TdgClinicalTrial TTD DrugBank GuideToPharmacology ChemblInteractions

  • ATALUREN   CFTR

    Interaction Score: 3.16

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    18722008 21233271 26840186 20622033


    Sources:
    PharmGKB

  • GALICAFTOR   CFTR

    Interaction Score: 1.89

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    None found


    Sources:
    TTD

  • BAMOCAFTOR   CFTR

    Interaction Score: 1.89

    Interaction Types & Directionality:
    n/a

    Interaction Info:
    Specific Action of the Ligand Binding
    Endogenous Drug? False
    Direct Interaction? False

    PMIDs:
    None found


    Sources:
    GuideToPharmacology

  • IOWH-032   CFTR

    Interaction Score: 1.89

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    None found


    Sources:
    TTD

  • QBW251   CFTR

    Interaction Score: 1.89

    Interaction Types & Directionality:
    activator (activating)

    Interaction Info:
    Direct Interaction yes
    Mechanism of Interaction Cystic fibrosis transmembrane conductance regulator activator

    PMIDs:
    None found


    Sources:
    ChemblInteractions

  • CHEMBL177991   CFTR

    Interaction Score: 0.95

    Interaction Types & Directionality:
    activator (activating)

    Interaction Info:
    Specific Action of the Ligand Potentiation
    Endogenous Drug? False

    PMIDs:
    None found


    Sources:
    GuideToPharmacology

  • CYSTEAMINE   CFTR

    Interaction Score: 0.95

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    27035618


    Sources:
    PharmGKB

  • LONIDAMINE   CFTR

    Interaction Score: 0.76

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    17490919


    Sources:
    DrugBank

  • GLYBURIDE   CFTR

    Interaction Score: 0.58

    Interaction Types & Directionality:
    antagonist (inhibitory)
    blocker (inhibitory)

    Interaction Info:
    Specific Action of the Ligand None
    Endogenous Drug? False

    PMIDs:
    12407077 12202948 12391048 14729151 15365090


    Sources:
    DrugBank GuideToPharmacology

  • COLFORSIN   CFTR

    Interaction Score: 0.32

    Interaction Types & Directionality:
    inhibitor (inhibitory)

    Interaction Info:

    PMIDs:
    15194485


    Sources:
    DrugBank

  • SCRIPTAID   CFTR

    Interaction Score: 0.32

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    19966789


    Sources:
    DTC

  • DEXFOSFOSERINE   CFTR

    Interaction Score: 0.29

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    10592235


    Sources:
    DrugBank

  • DEXIBUPROFEN   CFTR

    Interaction Score: 0.29

    Interaction Types & Directionality:
    inhibitor (inhibitory)

    Interaction Info:

    PMIDs:
    9710435


    Sources:
    DrugBank

  • BUMETANIDE   CFTR

    Interaction Score: 0.25

    Interaction Types & Directionality:
    antagonist (inhibitory)

    Interaction Info:

    PMIDs:
    9886939


    Sources:
    DrugBank

  • ENTINOSTAT   CFTR

    Interaction Score: 0.21

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    19966789


    Sources:
    DTC

  • TRICHOSTATIN   CFTR

    Interaction Score: 0.2

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    19966789


    Sources:
    DTC

  • GENISTEIN   CFTR

    Interaction Score: 0.15

    Interaction Types & Directionality:
    activator (activating)

    Interaction Info:
    Specific Action of the Ligand Potentiation
    Endogenous Drug? False

    PMIDs:
    27261451 12124395


    Sources:
    NCI PharmGKB GuideToPharmacology

  • CAPSAICIN   CFTR

    Interaction Score: 0.11

    Interaction Types & Directionality:
    activator (activating)

    Interaction Info:
    Specific Action of the Ligand Potentiation
    Endogenous Drug? False

    PMIDs:
    None found


    Sources:
    GuideToPharmacology

  • FELODIPINE   CFTR

    Interaction Score: 0.09

    Interaction Types & Directionality:
    activator (activating)

    Interaction Info:
    Specific Action of the Ligand Potentiation
    Endogenous Drug? False
    Direct Interaction? False

    PMIDs:
    None found


    Sources:
    GuideToPharmacology

  • IBUPROFEN   CFTR

    Interaction Score: 0.09

    Interaction Types & Directionality:
    inhibitor (inhibitory)

    Interaction Info:

    PMIDs:
    9710435


    Sources:
    DrugBank

  • NIMODIPINE   CFTR

    Interaction Score: 0.09

    Interaction Types & Directionality:
    activator (activating)

    Interaction Info:
    Endogenous Drug? False
    Specific Action of the Ligand Potentiation

    PMIDs:
    None found


    Sources:
    GuideToPharmacology

  • VORINOSTAT   CFTR

    Interaction Score: 0.08

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    19966789


    Sources:
    DTC

  • APIGENIN   CFTR

    Interaction Score: 0.07

    Interaction Types & Directionality:
    activator (activating)

    Interaction Info:
    Specific Action of the Ligand Potentiation
    Endogenous Drug? False

    PMIDs:
    None found


    Sources:
    GuideToPharmacology

  • ADENOSINE TRIPHOSPHATE   CFTR

    Interaction Score: 0.07

    Interaction Types & Directionality:
    cofactor (activating)

    Interaction Info:

    PMIDs:
    15623556


    Sources:
    DrugBank

  • CURCUMIN   CFTR

    Interaction Score: 0.05

    Interaction Types & Directionality:
    n/a

    Interaction Info:

    PMIDs:
    27007499


    Sources:
    PharmGKB

  • Ensembl: ENSG00000001626

    • Version: 101_38

    Alternate Names:
    CFTR Ensembl Gene Name

    Gene Info:
    Gene Biotype PROTEIN_CODING

    Publications:

  • HopkinsGroom: P13569

    • Version: 11-September-2012

    Alternate Names:
    CFTR Uniprot Gene Name
    CFTR_HUMAN Uniprot Id
    1080 Entrez Gene Id

    Gene Info:
    Human Readable Name DRUGGABLE GENOME
    Interpro Type Domain
    Uniprot Evidence 1: Evidence at protein level

    Gene Categories:
    ABC TRANSPORTER, DRUGGABLE GENOME

    Publications:

  • TdgClinicalTrial: P13569

    • Version: January-2014

    Alternate Names:
    CFTR Gene Symbol

    Gene Info:
    Target Class Transporters
    Target Subclass TC:3.A.1.202.1

    Publications:

  • RussLampel: ENSG00000001626

    • Version: 26-July-2011

    Alternate Names:
    ENSG00000001626 Ensembl Gene Id
    CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR (CFTR) (CAMP- DEPENDENT CHLORIDE CHANNEL). [SOURCE:UNIPROT/SWISSPROT;ACC:P13569] Description
    CFTR Display Id

    Gene Info:
    Human Readable Name DRUGGABLE GENOME

    Gene Categories:
    DRUGGABLE GENOME

    Publications:

  • GuideToPharmacology: 1080

    • Version: 29-September-2020

    Alternate Names:
    1884 HUGO Gene ID
    1884 HUGO Gene Symbol
    CF transmembrane conductance regulator HUGO Gene Name

    Gene Info:
    GuideToPharmacology Gene Category Name CFTR
    GuideToPharmacology Gene Category ID 129

    Gene Categories:
    ION CHANNEL

    Publications:

  • PharmGKB: CFTR

    • Version: 18-August-2020

    Alternate Names:
    PA109 PharmGKB ID

    Gene Info:

    Publications:
    Carlile GW et al., 2016, Latonduine Analogs Restore F508del-Cystic Fibrosis Transmembrane Conductance Regulator Trafficking through the Modulation of Poly-ADP Ribose Polymerase 3 and Poly-ADP Ribose Polymerase 16 Activity., Mol Pharmacol
    Boyle MP et al., 2014, A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial., Lancet Respir Med
    Taylor-Cousar JL et al., 2018, Lumacaftor/ivacaftor in patients with cystic fibrosis and advanced lung disease homozygous for F508del-CFTR., J Cyst Fibros

  • DrugBank: BE0001195

    • Version: 5.1.7

    Alternate Names:
    CFTR DrugBank Gene Name
    P13569 UniProt Accession
    1080 Entrez Gene Id

    Gene Info:

    Publications:
    Reddy et al., 1999, Bumetanide blocks CFTR GCl in the native sweat duct., Am. J. Physiol.
    Zhou et al., 2002, Probing an open CFTR pore with organic anion blockers., J. Gen. Physiol.
    Reddy et al., 2002, Effect of anion transport blockers on CFTR in the human sweat duct., J. Membr. Biol.

  • NCI: CFTR

    • Version: 14-September-2017

    Alternate Names:

    Gene Info:

    Publications:
    Saeed et al., 2008, Fenretinide prevents the development of osteoporosis in Cftr-KO mice., J. Cyst. Fibros.
    Derand et al., 2002, The cystic fibrosis mutation G551D alters the non-Michaelis-Menten behavior of the cystic fibrosis transmembrane conductance regulator (CFTR) channel and abolishes the inhibitory Genistein binding site., J. Biol. Chem.

  • DTC: CFTR

    • Version: 02-September-2020

    Alternate Names:

    Gene Info:

    Publications:
    Hutt DM et al., 2010, Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis., Nat Chem Biol
    Pesce E et al., 2015, Synthesis and structure-activity relationship of aminoarylthiazole derivatives as correctors of the chloride transport defect in cystic fibrosis., Eur J Med Chem

  • HingoraniCasas: ENSG00000001626

    • Version: 31-May-2017

    Alternate Names:
    ENSG00000001626 Gene Symbol
    CFTR Ensembl Id

    Gene Info:

    Gene Categories:
    DRUGGABLE GENOME

    Publications:

  • ChemblInteractions: ABCC7

    • Version: chembl_23

    Alternate Names:
    ABCC7 GENE_SYMBOL
    CFTR GENE_SYMBOL
    Cystic fibrosis transmembrane conductance regulator UNIPROT

    Gene Info:

    Publications:

  • Pharos: CFTR

    • Version: 03-September-2020

    Alternate Names:
    Cystic fibrosis transmembrane conductance regulator Gene Name
    P13569 UniProt ID

    Gene Info:

    Gene Categories:
    TRANSPORTER, ION CHANNEL

    Publications:

  • GO: CFTR

    • Version: 05-September-2020

    Alternate Names:
    ABCC7 GO Gene Synonym

    Gene Info:

    Gene Categories:
    CELL SURFACE, ABC TRANSPORTER

    Publications:

  • Tempus: CFTR

    • Version: 11-November-2018

    Alternate Names:
    CFTR Gene Symbol

    Gene Info:

    Gene Categories:
    CLINICALLY ACTIONABLE

    Publications:

  • TTD: cAMP-dependent chloride channel

    • Version: 2020.06.01

    Alternate Names:
    CFTR TTD Gene Abbreviation
    T55654 TTD Target ID

    Gene Info:

    Publications:

  • FDA: CFTR

    • Version: 04-September-2020

    Alternate Names:

    Gene Info:

    Publications:

Disclaimer: This resource is intended for purely research purposes. It should not be used for emergencies or medical or professional advice.

A finding of a drug-gene interaction or potentially druggable category does not necessarily indicate effectiveness (or lack thereof) of any drug or treatment regimen. A finding of no interaction or no potentially druggable category does not necessarily indicate lack of effectiveness of any drug or treatment regimen. Drug-gene interactions or potentially druggable categories are not presented in ranked order of potential or predicted efficacy.

The dgidb.org website does not provide any medical or healthcare products, services or advice, and is not for medical emergencies or urgent situations. IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY, CALL YOUR DOCTOR OR 911 IMMEDIATELY. Information contained on this website is not a substitute for a doctor's medical judgment or advice. We recommend that you discuss your specific, individual health concerns with your doctor or health care professional.

DGIdb (v4.2.0 - sha1 afd9f30b) • Last updated 2020-10-21