SummaryCystic fibrosis arises from the misfolding and premature degradation of CFTRΔF508, a Cl− ion channel with a single amino acid deletion. Yet, the quality-control machinery that selects CFTRΔF508 for degradation and the mechanism for its misfolding are not well defined. We identified an ER membrane-associated ubiquitin ligase complex containing the E3 RMA1, the E2 Ubc6e, and Derlin-1 that cooperates with the cytosolic Hsc70/CHIP E3 complex to triage CFTR and CFTRΔF508. Derlin-1 serves to retain CFTR in the ER membrane and interacts with RMA1 and Ubc6e to promote CFTR's proteasomal degradation. RMA1 is capable of recognizing folding defects in CFTRΔF508 coincident with translation, whereas the CHIP E3 appears to act posttranslationally....
The functional deficiency of the cystic fibrosis transmembrane conductance regulator (CFTR), a plasm...
Cystic Fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulat...
Cystic fibrosis (CF) is caused by mutations in the gene encoding of the cystic fibrosis transmembran...
Cystic fibrosis arises from the misfolding and premature degradation of CFTR Delta F508, a Cl- ion c...
SummaryCystic fibrosis arises from the misfolding and premature degradation of CFTRΔF508, a Cl− ion ...
Upon its identification in 1989, mutations in the gene encoding the cystic fibrosis transmembrane co...
AbstractMost cases of cystic fibrosis are caused by mutations that interfere with the biosynthetic f...
Defects in processing and trafficking of cystic fibrosis transmembrane conductance regulator. Cystic...
Cystic fibrosis transmembrane conductance regulator (CFTR) is a polytopic membrane protein that func...
PubMed ID: 19828134The most common mutation associated with cystic fibrosis is the deletion of pheny...
Biogenesis of cystic fibrosis transmembrane conductance regulator (CFTR) starts with its cotranslati...
A specialized Hsp40 protein, DNAJB12, was found to function on the cytoplasmic face of the ER with t...
AbstractMost cystic fibrosis (CF) patients carry the F508del mutation in the CFTR chloride channel p...
The ΔF508 mutant form of the cystic fibrosis transmembrane conductance regulator (ΔF508 CFTR) that i...
Background/Aims: The CFTR-Associated Ligand (CAL), a PDZ domain containing protein with two coiled-c...
The functional deficiency of the cystic fibrosis transmembrane conductance regulator (CFTR), a plasm...
Cystic Fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulat...
Cystic fibrosis (CF) is caused by mutations in the gene encoding of the cystic fibrosis transmembran...
Cystic fibrosis arises from the misfolding and premature degradation of CFTR Delta F508, a Cl- ion c...
SummaryCystic fibrosis arises from the misfolding and premature degradation of CFTRΔF508, a Cl− ion ...
Upon its identification in 1989, mutations in the gene encoding the cystic fibrosis transmembrane co...
AbstractMost cases of cystic fibrosis are caused by mutations that interfere with the biosynthetic f...
Defects in processing and trafficking of cystic fibrosis transmembrane conductance regulator. Cystic...
Cystic fibrosis transmembrane conductance regulator (CFTR) is a polytopic membrane protein that func...
PubMed ID: 19828134The most common mutation associated with cystic fibrosis is the deletion of pheny...
Biogenesis of cystic fibrosis transmembrane conductance regulator (CFTR) starts with its cotranslati...
A specialized Hsp40 protein, DNAJB12, was found to function on the cytoplasmic face of the ER with t...
AbstractMost cystic fibrosis (CF) patients carry the F508del mutation in the CFTR chloride channel p...
The ΔF508 mutant form of the cystic fibrosis transmembrane conductance regulator (ΔF508 CFTR) that i...
Background/Aims: The CFTR-Associated Ligand (CAL), a PDZ domain containing protein with two coiled-c...
The functional deficiency of the cystic fibrosis transmembrane conductance regulator (CFTR), a plasm...
Cystic Fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulat...
Cystic fibrosis (CF) is caused by mutations in the gene encoding of the cystic fibrosis transmembran...