› 8eig

Electron Microscopy
3.6Å resolution

The complex of phosphorylated human delta F508 cystic fibrosis transmembrane conductance regulator (CFTR) with elexacaftor (VX-445) and ATP/Mg

Released:
DOI: 10.2210/pdb8eig/pdb
PDB entry 8eig coloured by chain, front view.
PDB entry 8eig coloured by chain, side view.
PDB entry 8eig coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Molecular structures reveal synergistic rescue of Δ508 CFTR by Trikafta modulators.
Fiedorczuk K, Chen J
Science 378 284-290 (2022)
PMID: 36264792
Related structures: EMD-28155

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:
monomeric (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-146638 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Cystic fibrosis transmembrane conductance regulator Chain: A
Molecule details ›
Chain: A
Length: 1479 amino acids
Theoretical weight: 168.19 KDa
Source organism: Homo sapiens
Expression system: Homo sapiens
UniProt:
  • Canonical: P13569 (Residues: 1-1480; Coverage: 100%)
Gene names: ABCC7, CFTR
Sequence domains:

Ligands and Environments

4 bound ligands:
Ligand MG 2 x MG
Ligand ATP 2 x ATP
Ligand CLR 1 x CLR
Ligand WJX 1 x WJX
No modified residues

Experiments and Validation Details

Entry percentile scores
Resolution: 3.6Å
Relevant EMDB volumes: EMD-28155
Expression system: Homo sapiens

Quick links

Citations

7 review citations

CFTR Folding: From Structure and Proteostasis to Cystic Fibrosis Personalized Medicine.
McDonald et al. (2023)
6 more

1 mention without citation

In Silico and In Vitro Evaluation of the Mechanism of Action of Three VX809-Based Hybrid Derivatives as Correctors of the F508del CFTR Protein.
Baroni et al. (2023)