› 7svr

Electron Microscopy
3.9Å resolution

The complex of dephosphorylated human cystic fibrosis transmembrane conductance regulator (CFTR) and Lumacaftor (VX-809)

Released:
DOI: 10.2210/pdb7svr/pdb
PDB entry 7svr coloured by chain, front view.
PDB entry 7svr coloured by chain, side view.
PDB entry 7svr coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Mechanism of CFTR correction by type I folding correctors.
Fiedorczuk K, Chen J
Cell 185 158-168.e11 (2022)
PMID: 34995514
Related structures: EMD-25452

Function and Biology Details

Reaction catalysed: 
ATP + H(2)O + closed Cl(-) channel = ADP + phosphate + open Cl(-) channel
Biochemical function:
Biological process:
Cellular component:
Sequence domains:

Structure analysis Details

Assembly composition:
hetero dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-146649 (preferred)
Entry contents:
2 distinct polypeptide molecules
Macromolecules (2 distinct):
Cystic fibrosis transmembrane conductance regulator Chain: A
Molecule details ›
Chain: A
Length: 1480 amino acids
Theoretical weight: 168.34 KDa
Source organism: Homo sapiens
Expression system: Homo sapiens
UniProt:
  • Canonical: P13569 (Residues: 1-1480; Coverage: 100%)
Gene names: ABCC7, CFTR
Sequence domains:
Cystic fibrosis transmembrane conductance regulator Chain: B
Molecule details ›
Chain: B
Length: 19 amino acids
Theoretical weight: 1.64 KDa
Source organism: Homo sapiens
Expression system: Homo sapiens

Ligands and Environments

1 bound ligand:
Ligand VX8 1 x VX8
No modified residues

Experiments and Validation Details

Entry percentile scores
Resolution: 3.9Å
Relevant EMDB volumes: EMD-25452
Expression system: Homo sapiens

Quick links

Citations

9 review citations

Molecular mechanisms of cystic fibrosis - how mutations lead to misfunction and guide therapy.
Farinha et al. (2022)
8 more

4 mentions without citation

Molecular structures reveal synergistic rescue of Δ508 CFTR by Trikafta modulators.
Fiedorczuk et al. (2022)
3 more