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Literature summary for 5.6.1.6 extracted from

  • Bajmoczi, M.; Gadjeva, M.; Alper, S.L.; Pier, G.; Golan, D.E.
    Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa (2009), Am. J. Physiol. Cell Physiol., 297, C263-C277.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 5.6.1.6

Activating Compound

Activating Compound Comment Organism Structure
additional information treatment of IB3-1 cells with an aminoglycoside, e.g. G-418 or gentamicin, increases levels of W1282X-CFTR mRNA, with increased production of CFTR due to read-through of the W1282X stop mutation and parallel induction of cAMP-dependent Cl- efflux in treated cells Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expression of GFP-tagged CFTR in isogenic lung epithelial cell lines Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information construction of isogenic lung epithelial cell lines expressing either mutant or wild-type CFTR, development of a series of nominally isogenic, clonal bronchial epithelial cell lines stably expressing a 3 GFP-N-terminal labeled WT-CFTR, using as a parental cell line the IB3-1 cells initially obtained 4 from a cystic fibrosis patient with DELTAF508/W1282X cftr alleles, Pseudomonas aeruginosa infection phenotypes, overview Homo sapiens

General Stability

General Stability Organism
in cycloheximide-treated clone C9 cells, the expressed GFP-CFTR exhibits a half-life of 48-72 h Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
plasma membrane lipid raft cavaeolae Homo sapiens 5886
-

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
180000
-
 x * 180000, untagged CFTR, SDS-PAGE, x * 210000, GFP-tagged CFTR, SDS-PAGE Homo sapiens
210000
-
 x * 180000, untagged CFTR, SDS-PAGE, x * 210000, GFP-tagged CFTR, SDS-PAGE Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens wild-type CFTR functions as a cAMP-activated chloride channel that has an important role in ion transport across the apical surfaces of secretory epithelia, also requiring caveolin-1 spatially associated to CFTR. Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa in lungs. The cells reaction involves the rapid formation of detergent-resistant membrane microdomains, i.e. lipid rafts, containing CFTR. Enzyme defects lead to defective innate immunity and susceptibility to chronic lung infection with Pseudomonas aeruginosa in cystic fibrosis patients, overview ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
glycoprotein
-
 Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information wild-type CFTR functions as a cAMP-activated chloride channel that has an important role in ion transport across the apical surfaces of secretory epithelia, also requiring caveolin-1 spatially associated to CFTR. Cystic fibrosis transmembrane conductance regulator and caveolin-1 regulate epithelial cell internalization of Pseudomonas aeruginosa in lungs. The cells reaction involves the rapid formation of detergent-resistant membrane microdomains, i.e. lipid rafts, containing CFTR. Enzyme defects lead to defective innate immunity and susceptibility to chronic lung infection with Pseudomonas aeruginosa in cystic fibrosis patients, overview Homo sapiens ?
-
 ?

Subunits

Subunits Comment Organism
? x * 180000, untagged CFTR, SDS-PAGE, x * 210000, GFP-tagged CFTR, SDS-PAGE Homo sapiens

Synonyms

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