Send us a Message

Submit Data |  Help |  Video Tutorials |  News |  Publications |  Download |  REST API |  Citing RGD |  Contact   


The Chemical Entities of Biological Interest (ChEBI) ontology is downloaded weekly from EMBL-EBI at The data is made available under the Creative Commons License (CC BY 3.0, For more information see: Degtyarenko et al. (2008) ChEBI: a database and ontology for chemical entities of biological interest. Nucleic Acids Res. 36, D344–D350.

go back to main search page
Accession:CHEBI:66901 term browser browse the term
Definition:An aromatic amide obtained by formal condensation of the carboxy group of 4-oxo-1,4-dihydroquinoline-3-carboxylic acid with the amino group of 5-amino-2,4-di-tert-butylphenol. Used for the treatment of cystic fibrosis.
Synonyms:exact_synonym: N-(2,4-di-tert-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide
 related_synonym: Formula=C24H28N2O3;   InChI=1S/C24H28N2O3/c1-23(2,3)16-11-17(24(4,5)6)20(27)12-19(16)26-22(29)15-13-25-18-10-8-7-9-14(18)21(15)28/h7-13,27H,1-6H3,(H,25,28)(H,26,29);   InChIKey=PURKAOJPTOLRMP-UHFFFAOYSA-N;   Kalydeco;   SMILES=CC(C)(C)c1cc(c(NC(=O)c2c[nH]c3ccccc3c2=O)cc1O)C(C)(C)C;   VX-770;   ivacaftorum
 xref: CAS:873054-44-5;   Drug_Central:4228;   KEGG:D09916
 xref_mesh: MESH:C545203
 xref: PMID:22047557;   PMID:22293084;   PMID:22383668;   PMID:22499233;   PMID:22508750;   PMID:22543461;   PMID:22618984;   PMID:22698459;   PMID:22723294;   PMID:22739718;   PMID:22768130;   Reaxys:11821038;   Wikipedia:Ivacaftor

show annotations for term's descendants           Sort by:
ivacaftor term browser
Symbol Object Name Qualifiers Evidence Notes Source PubMed Reference(s) RGD Reference(s) Position
G Abcb11 ATP binding cassette subfamily B member 11 increases activity
multiple interactions
EXP ivacaftor results in increased activity of ABCB11 protein mutant form
ivacaftor inhibits the reaction [ABCB11 protein mutant form results in decreased transport of Taurocholic Acid]
CTD PMID:36142670 NCBI chr 3:54,016,854...54,112,797
Ensembl chr 3:54,017,127...54,112,730
JBrowse link
G Cftr CF transmembrane conductance regulator multiple interactions
increases activity
ISO [ivacaftor co-treated with Colforsin co-treated with lumacaftor] results in increased activity of CFTR protein mutant form; [ivacaftor co-treated with Colforsin] results in increased activity of CFTR protein; [ivacaftor results in increased activity of CFTR protein mutant form] which affects the transport of Sodium; [ivacaftor results in increased activity of CFTR protein mutant form] which results in increased secretion of Chlorides; ivacaftor inhibits the reaction [Vehicle Emissions results in decreased activity of CFTR protein]
ivacaftor results in increased activity of CFTR protein; ivacaftor results in increased activity of CFTR protein mutant form
CTD PMID:19846789 PMID:21083385 PMID:21602569 PMID:32374624 PMID:34601066 NCBI chr 4:46,561,269...46,728,759
Ensembl chr 4:46,560,885...46,728,756
JBrowse link

Term paths to the root
Path 1
Term Annotations click to browse term
  CHEBI ontology 19740
    role 19713
      biological role 19711
        biophysical role 13803
          membrane transport modulator 13639
            CFTR potentiator 120
              ivacaftor 2
                Orkambi 0
Path 2
Term Annotations click to browse term
  CHEBI ontology 19740
    subatomic particle 19738
      composite particle 19738
        hadron 19738
          baryon 19738
            nucleon 19738
              atomic nucleus 19738
                atom 19738
                  main group element atom 19686
                    p-block element atom 19686
                      carbon group element atom 19631
                        carbon atom 19627
                          organic molecular entity 19627
                            organic group 18846
                              organic divalent group 18832
                                organodiyl group 18832
                                  carbonyl group 18798
                                    carbonyl compound 18798
                                      carboxylic acid 18518
                                        carboacyl group 17656
                                          univalent carboacyl group 17656
                                            carbamoyl group 17497
                                              carboxamide 17497
                                                monocarboxylic acid amide 15562
                                                  ivacaftor 2
                                                    Orkambi 0
paths to the root