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Literature summary extracted from
- A small-molecule modulator interacts directly with DELTAPhe508-CFTR to modify its ATPase activity and conformational stability (2009), Mol. Pharmacol., 75, 1430-1438.
Activating Compound
| EC Number | Activating Compound | Comment | Organism | Structure |
|---|---|---|---|---|
| 5.6.1.6 | VRT-532 | i.e. 4-methyl-2-(5-phenyl-1H-pyrazol-3-yl)-phenol, the ATPase activity of the purified and reconstituted mutant DELTAPhe508-CFTR is directly modulated and ATP turnover is decreased by binding of VRT-532, but VRT-532 stimulates channel function of DELTAPhe508-CFTR in cells. VRT-532 binding induces a change in conformational stability of the C-terminal half of DELATPhe508-CFTR | Homo sapiens |  |
Cloned(Commentary)
| EC Number | Cloned (Comment) | Organism |
|---|---|---|
| 5.6.1.6 | expression of mutant DELTAPhe508 in BHK cells and as His-tagged protein in Spodoptera frugiperda Sf9 cells | Homo sapiens |
Protein Variants
| EC Number | Protein Variants | Comment | Organism |
|---|---|---|---|
| 5.6.1.6 | additional information | deletion of Phe508 constitutes the most prevalent of a number of mutations in CFTR that cause cystic fibrosis, mutation leads to CFTR misfolding and retention in the endoplasmic reticulum, as well as impaired channel activity. The biosynthetic defect can be partially overcome by small molecule correctors, once at the cell surface, small-molecule potentiators enhance the channel activity of DELTAPhe508 CFTR, VRT-532 exhibit both corrector and potentiator functions | Homo sapiens |
Inhibitors
| EC Number | Inhibitors | Comment | Organism | Structure |
|---|---|---|---|---|
| 5.6.1.6 | CFTRinh-172 | a specific CFTR inhibitor, inhibits ATPase activity of mutant DELTAPhe508-CFTR | Homo sapiens | |
| 5.6.1.6 | VRT-532 | i.e. 4-methyl-2-(5-phenyl-1H-pyrazol-3-yl)-phenol, the ATPase activity of the purified and reconstituted mutant DELTAPhe508-CFTR is directly modulated and ATP turnover is decreased by binding of VRT-532, but VRT-532 stimulates channel function of DELTAPhe508-CFTR in cells. VRT-532 binding induces a change in conformational stability of the C-terminal half of DELATPhe508-CFTR | Homo sapiens | |
KM Value [mM]
| EC Number | KM Value [mM] | KM Value Maximum [mM] | Substrate | Comment | Organism | Structure |
|---|---|---|---|---|---|---|
| 5.6.1.6 | 0.2 | - |
ATP | pH 7.5, 37°C, mutant DELTAPhe508-CFTR | Homo sapiens | |
Localization
| EC Number | Localization | Comment | Organism | GeneOntology No. | Textmining |
|---|---|---|---|---|---|
| 5.6.1.6 | membrane | - |
Homo sapiens | 16020 | - |
Metals/Ions
| EC Number | Metals/Ions | Comment | Organism | Structure |
|---|---|---|---|---|
| 5.6.1.6 | Mg2+ | - |
Homo sapiens | |
Natural Substrates/ Products (Substrates)
| EC Number | Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 5.6.1.6 | ATP + H2O + closed Cl- channel | Homo sapiens | the enzyme also shows ion channel activity | ADP + phosphate + open Cl- channel | - |
? | |
Organism
| EC Number | Organism | UniProt | Comment | Textmining |
|---|---|---|---|---|
| 5.6.1.6 | Homo sapiens | - |
- |
- |
Posttranslational Modification
| EC Number | Posttranslational Modification | Comment | Organism |
|---|---|---|---|
| 5.6.1.6 | phosphoprotein | - |
Homo sapiens |
Purification (Commentary)
| EC Number | Purification (Comment) | Organism |
|---|---|---|
| 5.6.1.6 | recombinant His-tagged mutant DELTAPhe508 from Sf9 cells by nickel affinity chromatography | Homo sapiens |
Substrates and Products (Substrate)
| EC Number | Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
|---|---|---|---|---|---|---|---|
| 5.6.1.6 | ATP + H2O + closed Cl- channel | - |
Homo sapiens | ADP + phosphate + open Cl- channel | - |
? | |
| 5.6.1.6 | ATP + H2O + closed Cl- channel | the enzyme also shows ion channel activity | Homo sapiens | ADP + phosphate + open Cl- channel | - |
? | |
Subunits
| EC Number | Subunits | Comment | Organism |
|---|---|---|---|
| 5.6.1.6 | ? | x * 140000-150000, recombinant mutant DELTAPhe508, SDS-PAGE | Homo sapiens |
Synonyms
| EC Number | Synonyms | Comment | Organism |
|---|---|---|---|
| 5.6.1.6 | CFTR | - |
Homo sapiens |
| 5.6.1.6 | cystic fibrosis transmembrane conductance regulator | - |
Homo sapiens |
Temperature Optimum [°C]
| EC Number | Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
|---|---|---|---|---|
| 5.6.1.6 | 27 | - |
ion channel assay at | Homo sapiens |
| 5.6.1.6 | 37 | - |
ATPase assay at | Homo sapiens |
pH Optimum
| EC Number | pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
|---|---|---|---|---|
| 5.6.1.6 | 7.4 | - |
ion channel assay at | Homo sapiens |
| 5.6.1.6 | 7.5 | - |
ATPase assay at | Homo sapiens |
IC50 Value
| EC Number | IC50 Value | IC50 Value Maximum | Comment | Organism | Inhibitor | Structure |
|---|---|---|---|---|---|---|
| 5.6.1.6 | 0.0026 | - |
pH 7.5, 37°C, inhibition of ATPase activity of mutant DELTAPhe508-CFTR | Homo sapiens | CFTRinh-172 | |
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