Most cystic fibrosis is caused by a deletion of a single residue (F508) in CFTR that disrupts the folding and biosynthetic maturation of the ion channel protein. Progress towards understanding the underlying mechanisms and overcoming the defect remain incomplete. Here we show that the thermal instability of human ΔF508 CFTR channel activity evident in both cell-attached membrane patches and planar phospholipid bilayers is not observed in corresponding mutant CFTRs of several non-mammalian species. These more stable orthologs are distinguished from their mammalian counterparts by the substitution of proline residues at several key dynamic locations in the first nucleotide domain (NBD1), including the structurally diverse region (SDR), the ga...
AbstractMost cystic fibrosis (CF) patients carry the F508del mutation in the CFTR chloride channel p...
Proteostasis (Balch et al. (2008) Science 319: 916) refers to the biology that maintains the proteom...
AbstractExperiments have demonstrated that the cystic fibrosis transmembrane conductance regulator p...
Most cystic fibrosis is caused by a deletion of a single residue (F508) in CFTR that disrupts the fo...
CFTR (ABCC7), unique among ABC exporters as an ion channel, regulates ion and fluid transport in epi...
CFTR (ABCC7), unique among ABC exporters as an ion channel, regulates ion and fluid transport in epi...
CFTR is unique among ABC transporters as the only one functioning as an ion channel and from a human...
CFTR is unique among ABC transporters as the only one functioning as an ion channel and from a human...
Deletion of Phe508 from cystic fibrosis transmembrane conductance regulator (CFTR) results in a temp...
The cystic fibrosis transmembrane conductance regulator (CFTR) epithelial anion channel is a large m...
The cystic fibrosis transmembrane conductance regulator (CFTR) epithelial anion channel is a large m...
The cystic fibrosis transmembrane conductance regulator (CFTR) requires dynamic fluctuations between...
The cystic fibrosis transmembrane conductance regulator (CFTR) requires dynamic fluctuations between...
AbstractBackgroundThe deletion of Phe508 in the first nucleotide-binding domain of the CFTR protein ...
Proteostasis (Balch et al. (2008) Science 319: 916) refers to the biology that maintains the proteom...
AbstractMost cystic fibrosis (CF) patients carry the F508del mutation in the CFTR chloride channel p...
Proteostasis (Balch et al. (2008) Science 319: 916) refers to the biology that maintains the proteom...
AbstractExperiments have demonstrated that the cystic fibrosis transmembrane conductance regulator p...
Most cystic fibrosis is caused by a deletion of a single residue (F508) in CFTR that disrupts the fo...
CFTR (ABCC7), unique among ABC exporters as an ion channel, regulates ion and fluid transport in epi...
CFTR (ABCC7), unique among ABC exporters as an ion channel, regulates ion and fluid transport in epi...
CFTR is unique among ABC transporters as the only one functioning as an ion channel and from a human...
CFTR is unique among ABC transporters as the only one functioning as an ion channel and from a human...
Deletion of Phe508 from cystic fibrosis transmembrane conductance regulator (CFTR) results in a temp...
The cystic fibrosis transmembrane conductance regulator (CFTR) epithelial anion channel is a large m...
The cystic fibrosis transmembrane conductance regulator (CFTR) epithelial anion channel is a large m...
The cystic fibrosis transmembrane conductance regulator (CFTR) requires dynamic fluctuations between...
The cystic fibrosis transmembrane conductance regulator (CFTR) requires dynamic fluctuations between...
AbstractBackgroundThe deletion of Phe508 in the first nucleotide-binding domain of the CFTR protein ...
Proteostasis (Balch et al. (2008) Science 319: 916) refers to the biology that maintains the proteom...
AbstractMost cystic fibrosis (CF) patients carry the F508del mutation in the CFTR chloride channel p...
Proteostasis (Balch et al. (2008) Science 319: 916) refers to the biology that maintains the proteom...
AbstractExperiments have demonstrated that the cystic fibrosis transmembrane conductance regulator p...