PDBe 2nsx

X-ray diffraction
2.11Å resolution

Structure of acid-beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease

Released:

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

Assemblies composition:
monomeric
homo tetramer
homo dimer (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
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, GC, GLUC
Sequence domains:
Structure domains:

Ligands and Environments


No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 23-ID-B
Spacegroup: P21
Unit cell:
a: 108.967Å b: 92.216Å c: 152.67Å
α: 90° β: 110.94° γ: 90°
R-values:
R R work R free
0.193 0.191 0.235
Expression system: Cricetulus griseus