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Literature summary for 5.6.1.6 extracted from

  • Xie, C.; Cao, X.; Chen, X.; Wang, D.; Zhang, W.K.; Sun, Y.; Hu, W.; Zhou, Z.; Wang, Y.; Huang, P.
    Mechanosensitivity of wild-type and G551D cystic fibrosis transmembrane conductance regulator (CFTR) controls regulatory volume decrease in simple epithelia (2016), FASEB J., 30, 1579-1589 .
    View publication on PubMedView publication on EuropePMC

Activating Compound

Activating Compound Comment Organism Structure
additional information the channel activity of both wild type and G551D mutant enzyme is directly stimulated by mechanical perturbation induced by cell swelling at the single-channel, cellular, and tissue levels. The enzyme is also activated by membrane stretch Mus musculus
additional information the channel activity of both wild type and G551D mutant enzyme is directly stimulated by mechanical perturbation induced by cell swelling at the single-channel, cellular, and tissue levels. The enzyme is also activated by membrane stretch Homo sapiens

Protein Variants

Protein Variants Comment Organism
G551D the mutation impairs ATP hydrolysis and thereby makes the enzyme refractory to cAMP stimulation Mus musculus
G551D the mutation impairs ATP hydrolysis and thereby makes the enzyme refractory to cAMP stimulation Homo sapiens

Inhibitors

Inhibitors Comment Organism Structure
CFTRinh-172 specific inhibitor Homo sapiens
CFTRinh-172 specific inhibitor Mus musculus
glybenclamide
-
Homo sapiens
glybenclamide
-
Mus musculus
GlyH-101 specific inhibitor Homo sapiens
GlyH-101 specific inhibitor Mus musculus
additional information 4,4'-diisothiocyano-2,29-stilbenedisulfonic acid and tamoxifen have little effect on enzyme activity Homo sapiens
additional information 4,4'-diisothiocyano-2,29-stilbenedisulfonic acid and tamoxifen have little effect on enzyme activity Mus musculus

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + H2O + closed Cl- channel Mus musculus
-
ADP + phosphate + open Cl- channel
-
?
ATP + H2O + closed Cl- channel Homo sapiens
-
ADP + phosphate + open Cl- channel
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-
Mus musculus
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
Calu-3 cell
-
Homo sapiens
-
duodenum
-
Mus musculus
-
small intestine
-
Mus musculus
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + H2O + closed Cl- channel
-
Mus musculus ADP + phosphate + open Cl- channel
-
?
ATP + H2O + closed Cl- channel
-
Homo sapiens ADP + phosphate + open Cl- channel
-
?

Synonyms

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

General Information

General Information Comment Organism
physiological function the channel activity of the enzyme is required for epithelial regulatory volume decrease Mus musculus
physiological function the channel activity of the enzyme is required for epithelial regulatory volume decrease Homo sapiens