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Literature summary for 5.6.1.6 extracted from

  • Cai, Z.; Palmai-Pallag, T.; Khuituan, P.; Mutolo, M.J.; Boinot, C.; Liu, B.; Scott-Ward, T.S.; Callebaut, I.; Harris, A.; Sheppard, D.N.
    Impact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating (2015), J. Physiol., 593, 2427-2446.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 5.6.1.6

Cloned(Commentary)

Cloned (Comment) Organism
phylogenetic analysis, recombinant expression of wild-type and mutant enzymes in CHO-K1 and HEK-293 cells Ovis aries

Protein Variants

Protein Variants Comment Organism
additional information the most common mutation involved in cystic fibrosis, F508del, has reduced impact on sheep CFTR function, single-channel behaviour analysis, phenotype, overview. Phylogenetic analysis of CFTR structure and function demonstrates that subtle changes in structure have pronounced effects on channel function and the consequences of the cystic fibrosis mutation F508del Ovis aries

Inhibitors

Inhibitors Comment Organism Structure
glibenclamide an open-channel blocker Ovis aries

Metals/Ions

Metals/Ions Comment Organism Structure
Mg2+ required Ovis aries

Organism

Organism UniProt Comment Textmining
Ovis aries Q00555
-
-

Source Tissue

Source Tissue Comment Organism Textmining
epithelium the enzyme is located predominantly in the apical membrane of epithelia lining ducts and tubes throughout the body Ovis aries
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information ATP gates ovine CFTR with greater affinity and efficacy than human CFTR Ovis aries ?
-
 ?

Synonyms

Synonyms Comment Organism
CFTR
-
 Ovis aries
cystic fibrosis transmembrane conductance regulator
-
 Ovis aries

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
 assay at Ovis aries

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
7.3
-
 assay at Ovis aries

General Information

General Information Comment Organism
malfunction malfunction of the cystic fibrosis transmembrane conductance regulator, a gated pathway for chloride movement, causes the common life-shortening genetic disease cystic fibrosis, development of a sheep model of cystic fibrosis. The most common mutation involved in cystic fibrosis, F508del, has reduced impact on sheep CFTR function Ovis aries
additional information the sheep enzyme is noticeably more active than human enzyme, while the most common mutation involved in cystic fibrosis, F508del, has reduced impact on sheep CFTR function, single-channel behaviour analysis, phenotype comparison, ATP gates ovine CFTR with greater affinity and efficacy than human CFTR. Three-dimensional enzyme modeling, overview Ovis aries
physiological function ovine enzyme is a Cl- channel with enhanced conductance and ATP-dependent gating. The enzyme forms a weakly inwardly rectifying Cl- channel regulated by PKA-dependent phosphorylation, inhibited by the open-channel blocker glibenclamide. The enzyme plays a pivotal role in transepithelial ion transport Ovis aries