› 2nsx

X-ray diffraction
2.11Å resolution

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

Released:
DOI: 10.2210/pdb2nsx/pdb
PDB entry 2nsx coloured by chain, front view.
PDB entry 2nsx coloured by chain, side view.
PDB entry 2nsx coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Structure of acid beta-glucosidase with pharmacological chaperone provides insight into Gaucher disease.
Lieberman RL, Wustman BA, Huertas P, Powe AC, Pine CW, Khanna R, Schlossmacher MG, Ringe D, Petsko GA
Nat Chem Biol 3 101-7 (2007)
PMID: 17187079

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
homo tetramer
homo dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-137378 (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 NAG 5 x NAG
Ligand SO4 18 x SO4
Ligand GOL 2 x GOL
Ligand IFM 2 x IFM
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

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Citations

45 review citations

Natural products to drugs: natural product-derived compounds in clinical trials.
Butler MS. (2008)
44 more

18 mentions without citation

Identification and characterization of ambroxol as an enzyme enhancement agent for Gaucher disease.
Maegawa et al. (2009)
17 more