› 3s5z

X-ray diffraction
2Å resolution

Pharmacological Chaperoning in Human alpha-Galactosidase

Released:
DOI: 10.2210/pdb3s5z/pdb
PDB entry 3s5z coloured by chain, front view.
PDB entry 3s5z coloured by chain, side view.
PDB entry 3s5z coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
The molecular basis of pharmacological chaperoning in human α-galactosidase.
Guce AI, Clark NE, Rogich JJ, Garman SC
Chem Biol 18 1521-6 (2011)
PMID: 22195554

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 (5 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: Nicotiana benthamiana
UniProt:
  • Canonical: P06280 (Residues: 32-429; Coverage: 100%)
Gene name: GLA
Sequence domains:
Structure domains:

Ligands and Environments

Carbohydrate polymer : NEW Components: NAG, BMA, XYP, MAN, FUC
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG, FUC
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG, BMA, XYP, MAN
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG, BMA, FUC
Carbohydrate polymer
4 bound ligands:
Ligand GLA 2 x GLA
Ligand GAL 2 x GAL
Ligand NAG 1 x NAG
Ligand GOL 1 x GOL
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: NSLS BEAMLINE X6A
Spacegroup: C2221
Unit cell:
a: 89.313Å b: 140.606Å c: 182.486Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.211 0.21 0.234
Expression system: Nicotiana benthamiana

Quick links

Citations

13 review citations

Pharmacological Chaperone Therapy: Preclinical Development, Clinical Translation, and Prospects for the Treatment of Lysosomal Storage Disorders.
Parenti et al. (2015)
12 more

4 mentions without citation

Second-Generation Pharmacological Chaperones: Beyond Inhibitors.
Tran et al. (2020)
3 more