Details of the Target

General Information of Target

Target ID LDTP02664
Target Name Cystic fibrosis transmembrane conductance regulator (CFTR)
Gene Name CFTR
Gene ID 1080
Synonyms
ABCC7; Cystic fibrosis transmembrane conductance regulator; CFTR; ATP-binding cassette sub-family C member 7; Channel conductance-controlling ATPase; EC 5.6.1.6; cAMP-dependent chloride channel
3D Structure
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2D Sequence (FASTA)
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3D Structure (PDB)
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Sequence
MQRSPLEKASVVSKLFFSWTRPILRKGYRQRLELSDIYQIPSVDSADNLSEKLEREWDRE
LASKKNPKLINALRRCFFWRFMFYGIFLYLGEVTKAVQPLLLGRIIASYDPDNKEERSIA
IYLGIGLCLLFIVRTLLLHPAIFGLHHIGMQMRIAMFSLIYKKTLKLSSRVLDKISIGQL
VSLLSNNLNKFDEGLALAHFVWIAPLQVALLMGLIWELLQASAFCGLGFLIVLALFQAGL
GRMMMKYRDQRAGKISERLVITSEMIENIQSVKAYCWEEAMEKMIENLRQTELKLTRKAA
YVRYFNSSAFFFSGFFVVFLSVLPYALIKGIILRKIFTTISFCIVLRMAVTRQFPWAVQT
WYDSLGAINKIQDFLQKQEYKTLEYNLTTTEVVMENVTAFWEEGFGELFEKAKQNNNNRK
TSNGDDSLFFSNFSLLGTPVLKDINFKIERGQLLAVAGSTGAGKTSLLMVIMGELEPSEG
KIKHSGRISFCSQFSWIMPGTIKENIIFGVSYDEYRYRSVIKACQLEEDISKFAEKDNIV
LGEGGITLSGGQRARISLARAVYKDADLYLLDSPFGYLDVLTEKEIFESCVCKLMANKTR
ILVTSKMEHLKKADKILILHEGSSYFYGTFSELQNLQPDFSSKLMGCDSFDQFSAERRNS
ILTETLHRFSLEGDAPVSWTETKKQSFKQTGEFGEKRKNSILNPINSIRKFSIVQKTPLQ
MNGIEEDSDEPLERRLSLVPDSEQGEAILPRISVISTGPTLQARRRQSVLNLMTHSVNQG
QNIHRKTTASTRKVSLAPQANLTELDIYSRRLSQETGLEISEEINEEDLKECFFDDMESI
PAVTTWNTYLRYITVHKSLIFVLIWCLVIFLAEVAASLVVLWLLGNTPLQDKGNSTHSRN
NSYAVIITSTSSYYVFYIYVGVADTLLAMGFFRGLPLVHTLITVSKILHHKMLHSVLQAP
MSTLNTLKAGGILNRFSKDIAILDDLLPLTIFDFIQLLLIVIGAIAVVAVLQPYIFVATV
PVIVAFIMLRAYFLQTSQQLKQLESEGRSPIFTHLVTSLKGLWTLRAFGRQPYFETLFHK
ALNLHTANWFLYLSTLRWFQMRIEMIFVIFFIAVTFISILTTGEGEGRVGIILTLAMNIM
STLQWAVNSSIDVDSLMRSVSRVFKFIDMPTEGKPTKSTKPYKNGQLSKVMIIENSHVKK
DDIWPSGGQMTVKDLTAKYTEGGNAILENISFSISPGQRVGLLGRTGSGKSTLLSAFLRL
LNTEGEIQIDGVSWDSITLQQWRKAFGVIPQKVFIFSGTFRKNLDPYEQWSDQEIWKVAD
EVGLRSVIEQFPGKLDFVLVDGGCVLSHGHKQLMCLARSVLSKAKILLLDEPSAHLDPVT
YQIIRRTLKQAFADCTVILCEHRIEAMLECQQFLVIEENKVRQYDSIQKLLNERSLFRQA
ISPSDRVKLFPHRNSSKCKSKPQIAALKEETEEEVQDTRL
Target Type
Successful
Target Bioclass
Enzyme
Family
ABC transporter superfamily, ABCC family, CFTR transporter (TC 3.A.1.202) subfamily
Subcellular location
Apical cell membrane
Function
Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis. Mediates the transport of chloride ions across the cell membrane . Channel activity is coupled to ATP hydrolysis. The ion channel is also permeable to HCO(3)(-); selectivity depends on the extracellular chloride concentration. Exerts its function also by modulating the activity of other ion channels and transporters. Plays an important role in airway fluid homeostasis. 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. Modulates the activity of the epithelial sodium channel (ENaC) complex, in part by regulating the cell surface expression of the ENaC complex. Inhibits the activity of the ENaC channel containing subunits SCNN1A, SCNN1B and SCNN1G. 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. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the transporter SLC4A7. Can inhibit the chloride channel activity of ANO1. Plays a role in the chloride and bicarbonate homeostasis during sperm epididymal maturation and capacitation.
TTD ID
T55654
Uniprot ID
P13569
DrugMap ID
TTRLZHP
Ensemble ID
ENST00000003084.11
HGNC ID
HGNC:1884
ChEMBL ID
CHEMBL4051

Target Site Mutations in Different Cell Lines

Cell line Mutation details Probe for labeling this protein in this cell
A2780 SNV: p.G314A; p.M952I .
AN3CA SNV: p.Y913H .
CFPAC1 SNV: p.Q814L .
COLO320 SNV: p.S1049G .
CORL88 SNV: p.L998Ter .
HCC15 SNV: p.G893R .
HCT15 SNV: p.L850F .
HEC1 Deletion: p.F17SfsTer8 .
HT115 SNV: p.R555I; p.F976L; p.E1329Ter .
KMCH1 SNV: p.S1235I .
MEWO SNV: p.E115K; p.M1105I .
MOLT4 SNV: p.Q250K IA-alkyne    Probe Info 
NALM6 Deletion: p.T1057del
Insertion: p.S1058RfsTer3		 .
OVISE SNV: p.A876P .
PC9 SNV: p.L1254Ter .
REH SNV: p.E1104Ter .
RH41 SNV: p.L986V .
RL952 SNV: p.E1104G .
SKMEL2 SNV: p.L365F .
SNU423 SNV: p.M1105V .
UACC257 SNV: p.G241E; p.I1295F .

Probe(s) Labeling This Target

ABPP Probe
Click To Hide/Show 1 Probe Related to This Target
Probe name Structure Binding Site(Ratio) Interaction ID Ref
IA-alkyne
 Probe Info  		N.A.  LDD0167  [1]

The Interaction Atlas With This Target

The Protein(s) Related To This Target

Enzyme
Click To Hide/Show 3 Protein(s) Interacting with This Target
Protein name Family Uniprot ID
Actin, cytoplasmic 1 (ACTB) Actin family P60709
Calpain-1 catalytic subunit (CAPN1) Peptidase C2 family P07384
E3 ubiquitin-protein ligase RNF5 (RNF5) RNF5 family Q99942
Transporter and channel
Click To Hide/Show 8 Protein(s) Interacting with This Target
Protein name Family Uniprot ID
B-cell receptor-associated protein 31 (BCAP31) BCAP29/BCAP31 family P51572
Calnexin (CANX) Calreticulin family P27824
Derlin-1 (DERL1) Derlin family Q9BUN8
Intermediate conductance calcium-activated potassium channel protein 4 (KCNN4) Potassium channel KCNN family O15554
Testis anion transporter 1 (SLC26A8) SLC26A/SulP transporter (TC 2.A.53) family Q96RN1
Golgi-associated PDZ and coiled-coil motif-containing protein (GOPC) . Q9HD26
Na(+)/H(+) exchange regulatory cofactor NHE-RF1 (NHERF1) . O14745
Na(+)/H(+) exchange regulatory cofactor NHE-RF2 (NHERF2) . Q15599
GPCR
Click To Hide/Show 1 Protein(s) Interacting with This Target
Protein name Family Uniprot ID
Lysophosphatidic acid receptor 2 (LPAR2) G-protein coupled receptor 1 family Q9HBW0
Other
Click To Hide/Show 3 Protein(s) Interacting with This Target
Protein name Family Uniprot ID
Golgi reassembly-stacking protein 2 (GORASP2) GORASP family Q9H8Y8
Keratin, type II cytoskeletal 8 (KRT8) Intermediate filament family P05787
Na(+)/H(+) exchange regulatory cofactor NHE-RF3 (PDZK1) NHER family Q5T2W1

The Drug(s) Related To This Target

Approved
Click To Hide/Show 10 Drug(s) Interacting with This Target
Drug Name Drug Type External ID
Bumetanide Small molecular drug DB00887
Crofelemer Small molecular drug D09VXM
Dexibuprofen Small molecular drug DB09213
Glyburide Small molecular drug DB01016
Ibuprofen Small molecular drug DB01050
Ivacaftor Small molecular drug D0W7WC
Tezacaftor Small molecular drug DB11712
Elexacaftor . DB15444
Lumacaftor + Ivacaftor . D0S9WB
Tezacaftor And Ivacaftor . D0E1HC
Phase 3
Click To Hide/Show 1 Drug(s) Interacting with This Target
Drug Name Drug Type External ID
Vx-661 Small molecular drug D0Z5HR
Phase 2
Click To Hide/Show 4 Drug(s) Interacting with This Target
Drug Name Drug Type External ID
Lumacaftor Small molecular drug D0X0CB
Abbv-2222 . D01DGZ
Glpg-1837 . D0E0JO
Iowh032 . D0N4PW
Phase 1
Click To Hide/Show 3 Drug(s) Interacting with This Target
Drug Name Drug Type External ID
Abbv-2451 . D0VT6X
Abbv-2737 . D0U0QC
Abbv-3067 . D0A1YF
Investigative
Click To Hide/Show 14 Drug(s) Interacting with This Target
Drug Name Drug Type External ID
Atp Small molecular drug DB00171
Cbiq Small molecular drug D04ZIP
Cftrinh-172 Small molecular drug D0P3ZK
Colforsin Small molecular drug DB02587
Dexfosfoserine Small molecular drug DB04522
Genistein Small molecular drug DB01645
Glyh-101 Small molecular drug D0S1FV
Phenylglycine-01 Small molecular drug D03PGV
Sulfonamide-01 Small molecular drug D05PDF
Uccf-029 Small molecular drug D0T9DI
Uccf-339 Small molecular drug D0C6YC
Uccf-853 Small molecular drug D0S0NB
Lonidamine . DB06266
Phosphoaminophosphonic Acid-adenylate Ester . DB04395

References

1 SP3-FAIMS Chemoproteomics for High-Coverage Profiling of the Human Cysteinome*. Chembiochem. 2021 May 14;22(10):1841-1851. doi: 10.1002/cbic.202000870. Epub 2021 Feb 18.
Mass spectrometry data entry: PXD023056 , PXD023059 , PXD023058 , PXD023057 , PXD023060