Target Information
Target General Infomation | |||||
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Target ID |
T55654
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Former ID |
TTDNS00605
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Target Name |
cystic fibrosis transmembrane conductance regulator
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Gene Name |
CFTR
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Synonyms |
ATPbinding cassette subfamily C member 7; Channel conductancecontrolling ATPase; Cystic fibrosis transmembrane conductance regulator; cAMPdependent chloride channel; CFTR
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Target Type |
Successful
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Disease | Cystic fibrosis [ICD9: 277; ICD10: E84] | ||||
Diarrhea [ICD9: 787.91; ICD10: A09, K59.1] | |||||
HIV-associated diarrhoea [ICD10: A09] | |||||
Function |
Involved in the transport of chloride ions. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the SLC4A7 transporter. Can inhibit the chloride channel activity of ANO1. Plays a role in the chloride and bicarbonate homeostasis during sperm epididymal maturation and capacitation.
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BioChemical Class |
Acid anhydrides hydrolase
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UniProt ID | |||||
EC Number |
EC 3.6.3.49
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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 |
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Drugs and Mode of Action | |||||
Drug(s) | Crofelemer | Drug Info | Approved | HIV-associated diarrhoea | [530433], [532210], [542477] |
Ivacaftor | Drug Info | Approved | Cystic fibrosis | [467679], [532210] | |
iOWH032 | Drug Info | Phase 2 | Diarrhea | [524714] | |
Lumacaftor | Drug Info | Phase 2 | Cystic fibrosis | [522612], [542504] | |
VX-661 | Drug Info | Phase 2 | Cystic fibrosis | [525275] | |
Activator | CBIQ | Drug Info | [543927] | ||
phenylglycine-01 | Drug Info | [543927] | |||
sulfonamide-01 | Drug Info | [543927] | |||
UCCF-029 | Drug Info | [543927] | |||
UCCF-339 | Drug Info | [543927] | |||
UCCF-853 | Drug Info | [543927] | |||
Inhibitor | CFTR inhibitors | Drug Info | [543927] | ||
iOWH032 | Drug Info | [531605] | |||
Blocker (channel blocker) | CFTRinh-172 | Drug Info | [543927] | ||
GlyH-101 | Drug Info | [543927] | |||
Modulator | Crofelemer | Drug Info | [530433], [532210] | ||
Ivacaftor | Drug Info | [551871] | |||
Regulator | Lumacaftor | Drug Info | [532863] | ||
VX-661 | Drug Info | [532904] | |||
Pathways | |||||
References | |||||
Ref 467679 | (http://www.guidetopharmacology.org/) Nucleic Acids Res. 2015 Oct 12. pii: gkv1037. The IUPHAR/BPS Guide to PHARMACOLOGY in 2016: towards curated quantitative interactions between 1300 protein targets and 6000 ligands. (Ligand id: 4342). | ||||
Ref 522612 | ClinicalTrials.gov (NCT00865904) Study of VX-809 in Cystic Fibrosis Subjects With the ???508-CFTR Gene Mutation. U.S. National Institutes of Health. | ||||
Ref 524714 | ClinicalTrials.gov (NCT02111304) Efficacy of iOWH032 in Dehydrating Cholera. U.S. National Institutes of Health. | ||||
Ref 525275 | ClinicalTrials.gov (NCT02508207) A Phase 2 Study to Evaluate Effects of VX-661/Ivacaftor on Lung and Extrapulmonary Systems in Subjects With Cystic Fibrosis, Homozygous for the F508del-CFTR Mutation. | ||||
Ref 530433 | Crofelemer, an antisecretory antidiarrheal proanthocyanidin oligomer extracted from Croton lechleri, targets two distinct intestinal chloride channels. Mol Pharmacol. 2010 Jan;77(1):69-78. | ||||
Ref 530433 | Crofelemer, an antisecretory antidiarrheal proanthocyanidin oligomer extracted from Croton lechleri, targets two distinct intestinal chloride channels. Mol Pharmacol. 2010 Jan;77(1):69-78. | ||||
Ref 531605 | Developing novel antisecretory drugs to treat infectious diarrhea. Future Med Chem. 2011 Aug;3(10):1317-25. | ||||
Ref 532863 | 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. 2014 Jul;2(7):527-38. |
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