General Information of the Protein
Protein ID
PT02042
Protein Name
Cystic fibrosis transmembrane conductance regulator
Secondarily
Protein Name
ATP-binding cassette sub-family C member 7
Channel conductance-controlling ATPase
cAMP-dependent chloride channel
Gene Name
CFTR
Secondarily
Gene Name
ABCC7
Sequence
MQRSPLEKASVVSKLFFSWTRPILRKGYRQRLELSDIYQIPSVDSADNLSEKLEREWDRELASKKNPKLINALRRCFFWRFMFYGIFLYLGEVTKAVQPLLLGRIIASYDPDNKEERSIAIYLGIGLCLLFIVRTLLLHPAIFGLHHIGMQMRIAMFSLIYKKTLKLSSRVLDKISIGQLVSLLSNNLNKFDEGLALAHFVWIAPLQVALLMGLIWELLQASAFCGLGFLIVLALFQAGLGRMMMKYRDQRAGKISERLVITSEMIENIQSVKAYCWEEAMEKMIENLRQTELKLTRKAAYVRYFNSSAFFFSGFFVVFLSVLPYALIKGIILRKIFTTISFCIVLRMAVTRQFPWAVQTWYDSLGAINKIQDFLQKQEYKTLEYNLTTTEVVMENVTAFWEEGFGELFEKAKQNNNNRKTSNGDDSLFFSNFSLLGTPVLKDINFKIERGQLLAVAGSTGAGKTSLLMVIMGELEPSEGKIKHSGRISFCSQFSWIMPGTIKENIIFGVSYDEYRYRSVIKACQLEEDISKFAEKDNIVLGEGGITLSGGQRARISLARAVYKDADLYLLDSPFGYLDVLTEKEIFESCVCKLMANKTRILVTSKMEHLKKADKILILHEGSSYFYGTFSELQNLQPDFSSKLMGCDSFDQFSAERRNSILTETLHRFSLEGDAPVSWTETKKQSFKQTGEFGEKRKNSILNPINSIRKFSIVQKTPLQMNGIEEDSDEPLERRLSLVPDSEQGEAILPRISVISTGPTLQARRRQSVLNLMTHSVNQGQNIHRKTTASTRKVSLAPQANLTELDIYSRRLSQETGLEISEEINEEDLKECFFDDMESIPAVTTWNTYLRYITVHKSLIFVLIWCLVIFLAEVAASLVVLWLLGNTPLQDKGNSTHSRNNSYAVIITSTSSYYVFYIYVGVADTLLAMGFFRGLPLVHTLITVSKILHHKMLHSVLQAPMSTLNTLKAGGILNRFSKDIAILDDLLPLTIFDFIQLLLIVIGAIAVVAVLQPYIFVATVPVIVAFIMLRAYFLQTSQQLKQLESEGRSPIFTHLVTSLKGLWTLRAFGRQPYFETLFHKALNLHTANWFLYLSTLRWFQMRIEMIFVIFFIAVTFISILTTGEGEGRVGIILTLAMNIMSTLQWAVNSSIDVDSLMRSVSRVFKFIDMPTEGKPTKSTKPYKNGQLSKVMIIENSHVKKDDIWPSGGQMTVKDLTAKYTEGGNAILENISFSISPGQRVGLLGRTGSGKSTLLSAFLRLLNTEGEIQIDGVSWDSITLQQWRKAFGVIPQKVFIFSGTFRKNLDPYEQWSDQEIWKVADEVGLRSVIEQFPGKLDFVLVDGGCVLSHGHKQLMCLARSVLSKAKILLLDEPSAHLDPVTYQIIRRTLKQAFADCTVILCEHRIEAMLECQQFLVIEENKVRQYDSIQKLLNERSLFRQAISPSDRVKLFPHRNSSKCKSKPQIAALKEETEEEVQDTRL
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Organism
Homo sapiens, Human
Protein Classification
Ion channel
>
Other ion channel
>
Chloride channel
>
Cystic fibrosis transmembrane conductance regulator
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:17182731, 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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Uniprot ID
Primary ID:
P13569

Secondarily ID:
Q20BG8
Q20BH2
Q2I0A1
Q2I102
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Ensembl ID
ENSG00000001626
HGNC ID
HGNC:1884
Subcellular Location
Apical cell membrane
Early endosome membrane
Cell membrane
Recycling endosome membrane
Endoplasmic reticulum membrane
Nucleus
Map of Molecular Bioactivity Related to the Protein
Map of Molecular Bioactivity Related to the Protein

Protein
Cell Line
Compound

Bioactivity Value:

<= 0.1 μM
> 0.1 μM and <= 10 μM
> 10 μM
Imprecise Activity
Table of Molecular Bioactivities Related to the Protein
Cell Line ID: CL000068 , A-549
Compound ID Compound Name Compound Formula
CP0061241
1-(6-benzyl-2-pyridin-2-yl-7,8-dihydro-5H-pyrido[4,3-d]pyrimidin-4-yl)piperidin-3-ol
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C24H27N5O
 1
1
EC50 = 400 nM
   TI
   LI
   LO
   TS
CP0403559
N-[2-[[6-[(4-fluorophenyl)methyl]-2-pyridin-2-yl-7,8-dihydro-5H-pyrido[4,3-d]pyrimidin-4-yl]amino]ethyl]acetamide
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C23H25FN6O
 1
1
EC50 = 400 nM
   TI
   LI
   LO
   TS
CP0409074
6-[(4-fluorophenyl)methyl]-2-pyridin-2-yl-N-(2-pyrrolidin-1-ylethyl)-7,8-dihydro-5H-pyrido[4,3-d]pyrimidin-4-amine
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C25H29FN6
 1
1
EC50 = 500 nM
   TI
   LI
   LO
   TS
CP0223510
N-[1-[6-[(4-chlorophenyl)methyl]-2-pyridin-2-yl-7,8-dihydro-5H-pyrido[4,3-d]pyrimidin-4-yl]piperidin-4-yl]acetamide
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C26H29ClN6O
 1
1
EC50 = 1900 nM
   TI
   LI
   LO
   TS
CP0230834
2-[4-[6-[(4-methoxyphenyl)methyl]-2-pyridin-2-yl-7,8-dihydro-5H-pyrido[4,3-d]pyrimidin-4-yl]piperazin-1-yl]acetamide
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C26H31N7O2
 1
1
EC50 = 10900 nM
   TI
   LI
   LO
   TS
CP0148604
2-[4-(6-benzyl-2-pyridin-2-yl-7,8-dihydro-5H-pyrido[4,3-d]pyrimidin-4-yl)piperazin-1-yl]-N,N-dimethylethanamine
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C27H35N7
 1
1
EC50 = 11700 nM
   TI
   LI
   LO
   TS
Biochemical Assays
Compound ID Compound Name Compound Formula
CP0088272
CHEBI:66901
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C24H28N2O3
 2
1 EC50 = 3 nM
2 EC50 = 236 nM
CP0272151
N-(1H-indol-6-yl)-4-oxo-1H-quinoline-3-carboxamide
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C18H13N3O2
 1
1 EC50 = 100 nM
CP0144592
N-[12-(5-chloro-2-methoxyanilino)-3,11-dithia-5,13-diazatricyclo[8.3.0.02,6]trideca-1(10),2(6),4,12-tetraen-4-yl]-2,2-dimethylpropanamide
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C21H23ClN4O2S2
 1
1 EC50 = 300 nM
CP0305495
1-(6-benzyl-2-pyridin-2-yl-7,8-dihydro-5H-pyrido[4,3-d]pyrimidin-4-yl)piperidine-4-carboxamide
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C25H28N6O
 1
1 EC50 = 1400 nM
CP0352404
N-[2-[(3-cyano-5,7-dimethylquinolin-2-yl)amino]ethyl]-3-methoxybenzamide
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C22H22N4O2
 2
1 EC50 = 1500 nM
2 EC50 = 5900 nM
CP0497612
N-[5-[2-(5-chloro-2-methoxyanilino)-5-[4-(hydroxymethyl)triazol-1-yl]-1,3-thiazol-4-yl]-4-methyl-1,3-thiazol-2-yl]-2,2-dimethylpropanamide
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C22H24ClN7O3S2
 1
1 EC50 = 1600 nM
CP0295398
N,N-dibenzyl-4-oxo-1H-quinoline-3-carboxamide
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C24H20N2O2
 1
1 EC50 = 2100 nM
CP0148603
Lumacaftor
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C24H18F2N2O5
 2
1 EC50 = 2600 nM
2 Kd = 47800 nM
CP0644629
1-(2,2-difluorobenzo[d][1,3]dioxol-5-yl)-N-((2R,4R)-2-(3,4-dimethoxyphenyl)-7-methoxychroman-4-yl)cyclopropanecarboxamide
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C29H27F2NO7
 1
1 EC50 ~ 28 nM
CP0047295
N-[5-[2-(5-chloro-2-methoxyanilino)-1,3-thiazol-4-yl]-4-methyl-1,3-thiazol-2-yl]benzamide
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C21H17ClN4O2S2
 1
1 EC50 <= 2800 nM
CP0643856
1-(2,2-Difluoro-1,3-benzodioxol-5-yl)-N-[(2S,4S)-2-(3,4-dimethoxyphenyl)-7-methoxy-3,4-dihydro-2H-chromen-4-yl]cyclopropanecarboxamide
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C29H27F2NO7
 1
1 EC50 > 3000 nM
CP0071868
4-((4-oxo-2-thioxo-3-(3-(trifluoromethyl)phenyl)thiazolidin-5-ylidene)methyl)benzoic acid
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C18H10F3NO3S2
 1
1 IC50 = 300 nM
Clinical Information about the Protein
Target 1 ( cAMP-dependent chloride channel (CFTR) )
Target Type Successful Target
Disease 2 Target-related Diseases  2
1 Cystic fibrosis [ICD-11: CA25]
2 Chronic obstructive pulmonary disease [ICD-11: CA22]
Approved Drug(s) 1 Approved Drug  1
1 Ivacaftor Approved
Cystic fibrosis
Clinical Trial Drug(s) 5 Clinical Trial Drugs  5
1 VX-661 Phase 3
Cystic fibrosis
2 ABBV-2222 Phase 2
Cystic fibrosis
3 GLPG-1837 Phase 2
Cystic fibrosis
4 Lumacaftor Phase 2
Cystic fibrosis
5 QBW251 Phase 2
Chronic obstructive pulmonary disease