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Literature summary for 5.6.1.6 extracted from

  • He, L.; Aleksandrov, A.A.; Serohijos, A.W.; Hegedus, T.; Aleksandrov, L.A.; Cui, L.; Dokholyan, N.V.; Riordan, J.R.
    Multiple membrane-cytoplasmic domain contacts in the cystic fibrosis transmembrane conductance regulator (CFTR) mediate regulation of channel gating (2008), J. Biol. Chem., 283, 26383-26390.
    View publication on PubMedView publication on EuropePMC

Activating Compound

Activating Compound Comment Organism Structure
3-isobutyl-1-methylxanthine
-
Homo sapiens
DiBu-cAMP
-
Homo sapiens
forskolin
-
Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expression of wild-type and mutant enzymes in HEK-293 cells Homo sapiens

Protein Variants

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/ Products (Substrates)

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

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

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 and Products (Substrate)

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

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

Synonyms Comment Organism
CFTR
-
Homo sapiens
cystic fibrosis transmembrane conductance regulator
-
Homo sapiens