› 1y7v

X-ray diffraction
2.4Å resolution

X-ray structure of human acid-beta-glucosidase covalently bound to conduritol B epoxide

Released:
DOI: 10.2210/pdb1y7v/pdb
PDB entry 1y7v coloured by chain, front view.
PDB entry 1y7v coloured by chain, side view.
PDB entry 1y7v coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
X-ray structure of human acid-beta-glucosidase covalently bound to conduritol-B-epoxide. Implications for Gaucher disease.
Premkumar L, Sawkar AR, Boldin-Adamsky S, Toker L, Silman I, Kelly JW, Futerman AH, Sussman JL
J Biol Chem 280 23815-9 (2005)
PMID: 15817452

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 domains:

Structure analysis Details

Assemblies composition:
monomeric (preferred)
homo tetramer
homo dimer
Assembly name:
PDBe Complex ID:
PDB-CPX-137377 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (2 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
2 bound ligands:
Ligand SO4 13 x SO4
Ligand INS 2 x INS
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: RIGAKU RU300
Spacegroup: C2221
Unit cell:
a: 104.49Å b: 285.61Å c: 91.51Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.244 0.242 0.28
Expression system: Cricetulus griseus

Quick links

Citations

9 review citations

Gaucher disease: mutation and polymorphism spectrum in the glucocerebrosidase gene (GBA).
Hruska et al. (2008)
8 more

2 mentions without citation

X-ray and biochemical analysis of N370S mutant human acid β-glucosidase.
Wei et al. (2011)
1 more