› 2j25

X-ray diffraction
2.9Å resolution

Partially deglycosylated glucoceramidase

Released:
DOI: 10.2210/pdb2j25/pdb
PDB 2j25 coloured by chain and viewed from the front.
PDB 2j25 coloured by chain and viewed from the side.
PDB 2j25 coloured by chain and viewed from the top.
Source organism: Homo sapiens
Primary publication:
Structural comparison of differently glycosylated forms of acid-beta-glucosidase, the defective enzyme in Gaucher disease.
Brumshtein B, Wormald MR, Silman I, Futerman AH, Sussman JL
Acta Crystallogr. D Biol. Crystallogr. 62 1458-65 (2006)
PMID: 17139081

Function and Biology Details

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

Structure analysis Details

Assembly composition:
monomeric (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (4 distinct):
Lysosomal acid glucosylceramidase Chains: A, B
Molecule details ›
Chains: A, B
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

Carbohydrate polymer : NEW Components: NAG
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG, BMA, MAN
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG, BMA
Carbohydrate polymer
2 bound ligands:
Ligand NAG 1 x NAG
Ligand SO4 17 x SO4
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: ESRF BEAMLINE ID23-1
Spacegroup: C2221
Unit cell:
a: 108.626Å b: 280.821Å c: 91.064Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.218 0.215 0.273
Expression system: Cricetulus griseus

Quick links

Citations

9 review citations

Metabolism of Glycosphingolipids and Their Role in the Pathophysiology of Lysosomal Storage Disorders.
Ryckman et al. (2020)
8 more

6 mentions without citation

Characterization of gene-activated human acid-beta-glucosidase: crystal structure, glycan composition, and internalization into macrophages.
Brumshtein et al. (2010)
5 more