Activating Compound | Comment | Organism | Structure |
---|---|---|---|
calnexin | Dependence on calnexin for proper assembly of CFTRs 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 (Comment) | Organism |
---|---|
expression of wild-type and mutant DELTAF508 in HEK-293 cells, and co-expression of CFTR halves | Homo sapiens |
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 | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
membrane | - |
Homo sapiens | 16020 | - |
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 | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Posttranslational Modification | Comment | Organism |
---|---|---|
glycoprotein | glycosylation analysis using endoglycosidase H and peptide N-glycosidase F digestion | Homo sapiens |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
epithelial cell | apical surface of epithelial cells that line ducts of glands and airways | Homo sapiens | - |
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 | 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 | 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 |