› 4kgl

X-ray diffraction
2.7Å resolution

Crystal structure of human alpha-L-iduronidase complex with [2R,3R,4R,5S]-2-carboxy-3,4,5-trihydroxy-piperidine

Released:
DOI: 10.2210/pdb4kgl/pdb
PDB entry 4kgl coloured by chain, front view.
PDB entry 4kgl coloured by chain, side view.
PDB entry 4kgl coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Insights into mucopolysaccharidosis I from the structure and action of α-L-iduronidase.
Bie H, Yin J, He X, Kermode AR, Goddard-Borger ED, Withers SG, James MN
Nat Chem Biol 9 739-45 (2013)
PMID: 24036510

Function and Biology Details

Reaction catalysed: 
Hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate
Biochemical function:
Biological process:
Cellular component:
  • not assigned
Sequence domains:

Structure analysis Details

Assembly composition:
monomeric (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-152929 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (4 distinct):
Alpha-L-iduronidase Chains: A, B
Molecule details ›
Chains: A, B
Length: 627 amino acids
Theoretical weight: 70.05 KDa
Source organism: Homo sapiens
Expression system: Arabidopsis thaliana
UniProt:
  • Canonical: P35475 (Residues: 27-653; Coverage: 100%)
Gene name: IDUA
Sequence domains:
Structure domains:

Ligands and Environments

Carbohydrate polymer : NEW Components: NAG
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG, BMA, MAN
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG, BMA, MAN
Carbohydrate polymer
4 bound ligands:
Ligand NAG 2 x NAG
Ligand GOL 5 x GOL
Ligand IDJ 2 x IDJ
Ligand TLA 1 x TLA
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: SSRL BEAMLINE BL11-1
Spacegroup: R3
Unit cell:
a: 259.359Å b: 259.359Å c: 71.568Å
α: 90° β: 90° γ: 120°
R-values:
R R work R free
0.206 0.204 0.254
Expression system: Arabidopsis thaliana

Quick links

Citations

8 review citations

Dissecting conformational contributions to glycosidase catalysis and inhibition.
Speciale et al. (2014)
7 more

2 mentions without citation

Insights into mucopolysaccharidosis I from the structure and action of α-L-iduronidase.
Bie et al. (2013)
1 more