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Literature summary for 5.6.1.6 extracted from

  • Rosser, M.F.; Grove, D.E.; Chen, L.; Cyr, D.M.
    Assembly and misassembly of cystic fibrosis transmembrane conductance regulator: folding defects caused by deletion of F508 occur before and after the calnexin-dependent association of membrane spanning domain (MSD) 1 and MSD2 (2008), Mol. Biol. Cell, 19, 4570-4579.
    View publication on PubMedView publication on EuropePMC

Activating Compound

Activating Compound Comment Organism Structure
calnexin Dependence on calnexin for proper assembly of CFTR’s membrane spanning domains exists, also efficient folding of NBD2 is dependent upon calnexin binding to CFTR, but calnexin is not essential for wild-type CFTR or mutant CFTR DELTAF508 degradation Homo sapiens

Cloned(Commentary)

Cloned (Comment) Organism
expression of wild-type and mutant DELTAF508 in HEK-293 cells, and co-expression of CFTR halves Homo sapiens

Protein Variants

Protein Variants Comment Organism
additional information enzyme knockout by RNAi, folding defects caused by deletion of F508 that occur before and after the calnexin-dependent association of membrane spanning domains MSD1 and MSD2, mechanism, overview Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
membrane
-
Homo sapiens 16020
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
ATP + H2O + closed Cl- channel Homo sapiens CFTR functions as an ATP-gated Cl- channel that is critical for proper hydration of the mucosal layer that lines lung airways. Individuals who inherit two mutant forms of CFTR have exceedingly viscous mucous and, due to chronic lung infections, develop cystic fibrosis and often die from lung failure ADP + phosphate + open Cl- channel
-
?
additional information Homo sapiens cytosolic 70-kDa heat shock protein and endoplasmic reticulum-localized calnexin are chaperones that facilitate CFTR biogenesis. Hsp70 functions in both the cotranslational folding and posttranslational degradation of CFTR ?
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
glycoprotein glycosylation analysis using endoglycosidase H and peptide N-glycosidase F digestion Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining
epithelial cell apical surface of epithelial cells that line ducts of glands and airways Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
ATP + H2O + closed Cl- channel CFTR functions as an ATP-gated Cl- channel that is critical for proper hydration of the mucosal layer that lines lung airways. Individuals who inherit two mutant forms of CFTR have exceedingly viscous mucous and, due to chronic lung infections, develop cystic fibrosis and often die from lung failure Homo sapiens ADP + phosphate + open Cl- channel
-
?
additional information cytosolic 70-kDa heat shock protein and endoplasmic reticulum-localized calnexin are chaperones that facilitate CFTR biogenesis. Hsp70 functions in both the cotranslational folding and posttranslational degradation of CFTR Homo sapiens ?
-
?

Subunits

Subunits Comment Organism
More CFTR is a polytopic membrane protein that functions as a Cl- channel and consists of two membrane spanning domains, two cytosolic nucleotide binding domains, and a cytosolic regulatory domain. The assembly of CFTR into an ion channel is complicated because it requires the coordinated folding and assembly of its membrane and cytoplasmic domains into a functional unit. Misfolding of CFTR and analysis by trypsin proteolysis patterns of misfolded CFTR, model of calnexin action in CFTR folding, overview Homo sapiens

Synonyms

Synonyms Comment Organism
CFTR
-
Homo sapiens
cystic fibrosis transmembrane conductance regulator
-
Homo sapiens
More CFTR is a member of the ATP-binding cassette (ABC) transporter superfamily Homo sapiens