Activating Compound | Comment | Organism | Structure |
---|---|---|---|
3-isobutyl-1-methylxanthine | - |
Homo sapiens | |
DiBu-cAMP | - |
Homo sapiens | |
forskolin | - |
Homo sapiens |
Cloned (Comment) | Organism |
---|---|
expression of wild-type and mutant enzymes in HEK-293 cells | Homo sapiens |
Protein Variants | Comment | Organism |
---|---|---|
E543C/T996C | cross-linking between residues T996C and E543C at the CL3/NBD1 interface rapidly and reversibly arrests channel gating | Homo sapiens |
L172C/D1341C | the cross-linking between L172C of CL1 and D1341C of NBD2 rapidly and reversibly inhibited channel gating | Homo sapiens |
additional information | construction of Cys-less mutant CFTR. The most common naturally occurring mutation, deletion of phenylalanine 508, and many other disease-associated mutations occur in the nucleotide binding domains and the cytoplasmic loops of the membrane-spanning domains | Homo sapiens |
V171C/L408C | the mutation has essentially no influence on gating | Homo sapiens |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
additional information | Homo sapiens | the cystic fibrosis transmembrane conductance regulator is a unique ATP-binding cassette ion channel mutated in patients with cystic fibrosis | ? | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
phosphoprotein | control of CFTR channel activity is modulated by the phosphorylation of the R domain by protein kinase A, which allows the regulation of gating by ATP binding at the NBD1/NBD2 interface | Homo sapiens |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
ATP + H2O + closed Cl- channel | stable binding of ATP at nucleotide binding site NBD1 and binding and hydrolysis of ATP at NBD2, together with R domain phosphorylation, may alter allosteric interactions between these domains and impact the channel gating cycle | Homo sapiens | ADP + phosphate + open Cl- channel | - |
? | |
additional information | the cystic fibrosis transmembrane conductance regulator is a unique ATP-binding cassette ion channel mutated in patients with cystic fibrosis | Homo sapiens | ? | - |
? |
Subunits | Comment | Organism |
---|---|---|
More | the interdomain contacts comprise aromatic clusters important for stabilization of the interfaces and also involve the Q-loops and X-loops that are in close proximity to the ATP binding sites. Aromatic clusters mediate domain-swapping interactions, structure modelling, overview | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
CFTR | - |
Homo sapiens |
cystic fibrosis transmembrane conductance regulator | - |
Homo sapiens |