› 3gxp

X-ray diffraction
2.2Å resolution

Crystal structure of acid-alpha-galactosidase A complexed with galactose at pH 4.5

Released:
DOI: 10.2210/pdb3gxp/pdb
PDB entry 3gxp coloured by chain, front view.
PDB entry 3gxp coloured by chain, side view.
PDB entry 3gxp 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

Reaction catalysed: 
Hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids
Biochemical function:
Biological process:
Cellular component:
Sequence domains:

Structure analysis Details

Assembly composition:
homo dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-138990 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (3 distinct):
Alpha-galactosidase A Chains: A, B
Molecule details ›
Chains: A, B
Length: 398 amino acids
Theoretical weight: 45.39 KDa
Source organism: Homo sapiens
Expression system: Cricetulus griseus
UniProt:
  • Canonical: P06280 (Residues: 32-429; Coverage: 100%)
Gene name: GLA
Sequence domains:
Structure domains:

Ligands and Environments

Carbohydrate polymer : NEW Components: NAG, MAN
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG
Carbohydrate polymer
5 bound ligands:
Ligand SO4 4 x SO4
Ligand GLA 2 x GLA
Ligand TAM 1 x TAM
Ligand NAG 2 x NAG
Ligand MAN 2 x MAN
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 23-ID-D
Spacegroup: P3221
Unit cell:
a: 89.51Å b: 89.51Å c: 215.927Å
α: 90° β: 90° γ: 120°
R-values:
R R work R free
0.204 0.201 0.265
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

7 mentions without citation

Fabry Disease: Molecular Basis, Pathophysiology, Diagnostics and Potential Therapeutic Directions.
Kok et al. (2021)
6 more