› 3rik

X-ray diffraction
2.48Å resolution

The acid beta-glucosidase active site exhibits plasticity in binding 3,4,5,6-tetrahydroxyazepane-based inhibitors: implications for pharmacological chaperone design for gaucher disease

Released:
DOI: 10.2210/pdb3rik/pdb
PDB entry 3rik coloured by chain, front view.
PDB entry 3rik coloured by chain, side view.
PDB entry 3rik coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Binding of 3,4,5,6-tetrahydroxyazepanes to the acid-β-glucosidase active site: implications for pharmacological chaperone design for Gaucher disease.
Orwig SD, Tan YL, Grimster NP, Yu Z, Powers ET, Kelly JW, Lieberman RL
Biochemistry 50 10647-57 (2011)
PMID: 22047104

Function and Biology Details

Reactions catalysed: 
D-glucosyl-N-acylsphingosine + H(2)O = D-glucose + N-acylsphingosine
D-galactosyl-N-acylsphingosine + H(2)O = D-galactose + N-acylsphingosine
Biochemical function:
Biological process:
Cellular component:
Sequence domains:

Structure analysis Details

Assembly composition:
homo dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-137378 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
Lysosomal acid glucosylceramidase Chains: A, B, C, D
Molecule details ›
Chains: A, B, C, D
Length: 497 amino acids
Theoretical weight: 55.64 KDa
Source organism: Homo sapiens
Expression system: Cricetulus griseus
UniProt:
  • Canonical: P04062 (Residues: 40-536; Coverage: 100%)
Gene names: GBA, GBA1, GC, GLUC
Sequence domains:
Structure domains:

Ligands and Environments

3 bound ligands:
Ligand SO4 34 x SO4
Ligand NAG 4 x NAG
Ligand 3RI 2 x 3RI
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 22-BM
Spacegroup: P21
Unit cell:
a: 107.98Å b: 91.553Å c: 152.213Å
α: 90° β: 110.7° γ: 90°
R-values:
R R work R free
0.165 0.162 0.225
Expression system: Cricetulus griseus

Quick links

Citations

4 review citations

Recent developments in targeting protein misfolding diseases.
Denny et al. (2013)
3 more

4 mentions without citation

Binding of 3,4,5,6-tetrahydroxyazepanes to the acid-β-glucosidase active site: implications for pharmacological chaperone design for Gaucher disease.
Orwig et al. (2011)
3 more