PDBe 3ril

X-ray diffraction
2.4Å 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


Function and Biology Details

Reaction catalysed:
D-glucosyl-N-acylsphingosine + H(2)O = D-glucose + N-acylsphingosine
Biochemical function:
Biological process:
Cellular component:

Structure analysis Details

Assembly composition:
homo dimer (preferred)
Entry contents:
1 distinct polypeptide molecule
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
  • Canonical: P04062 (Residues: 40-536; Coverage: 100%)
Gene names: GBA, GC, GLUC
Sequence domains:
Structure domains:

Ligands and Environments

3 bound ligands:

No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 22-ID
Spacegroup: P21
Unit cell:
a: 109.217Å b: 91.425Å c: 152.659Å
α: 90° β: 110.95° γ: 90°
R R work R free
0.174 0.171 0.232
Expression system: Cricetulus griseus