› 3gxf

X-ray diffraction
2.4Å resolution

Crystal structure of acid-beta-glucosidase with isofagomine at neutral pH

Released:
DOI: 10.2210/pdb3gxf/pdb
PDB entry 3gxf coloured by chain, front view.
PDB entry 3gxf coloured by chain, side view.
PDB entry 3gxf coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability.
Lieberman RL, D'aquino JA, Ringe D, Petsko GA
Biochemistry 48 4816-27 (2009)
PMID: 19374450

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 tetramer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-137379 (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

4 bound ligands:
Ligand PO4 64 x PO4
Ligand NAG 4 x NAG
Ligand GOL 5 x GOL
Ligand IFM 2 x IFM
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 23-ID-D
Spacegroup: P21
Unit cell:
a: 110.311Å b: 92.013Å c: 152.362Å
α: 90° β: 111.21° γ: 90°
R-values:
R R work R free
0.182 0.178 0.245
Expression system: Cricetulus griseus

Quick links

Citations

22 review citations

Treating lysosomal storage diseases with pharmacological chaperones: from concept to clinics.
Parenti G. (2009)
21 more

5 mentions without citation

Effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability.
Lieberman et al. (2009)
4 more