› 4xwh

X-ray diffraction
2.32Å resolution

Crystal structure of the human N-acetyl-alpha-glucosaminidase

Released:
DOI: 10.2210/pdb4xwh/pdb
PDB entry 4xwh coloured by chain, front view.
PDB entry 4xwh coloured by chain, side view.
PDB entry 4xwh coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Structural characterization of the α-N-acetylglucosaminidase, a key enzyme in the pathogenesis of Sanfilippo syndrome B.
Birrane G, Dassier AL, Romashko A, Lundberg D, Holmes K, Cottle T, Norton AW, Zhang B, Concino MF, Meiyappan M
J Struct Biol 205 65-71 (2019)
PMID: 30802506

Function and Biology Details

Reaction catalysed: 
Hydrolysis of terminal non-reducing N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides
Biochemical function:
Biological process:
Cellular component:
Sequence domains:

Structure analysis Details

Assembly composition:
monomeric (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-157107 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (3 distinct):
Alpha-N-acetylglucosaminidase Chain: A
Molecule details ›
Chain: A
Length: 720 amino acids
Theoretical weight: 80.5 KDa
Source organism: Homo sapiens
Expression system: Homo sapiens
UniProt:
  • Canonical: P54802 (Residues: 24-743; Coverage: 100%)
Gene names: NAGLU, UFHSD1
Sequence domains:

Ligands and Environments

Carbohydrate polymer : NEW Components: NAG, BMA
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG
Carbohydrate polymer
3 bound ligands:
Ligand NAG 3 x NAG
Ligand GOL 3 x GOL
Ligand XYL 2 x XYL
1 modified residue:
Ligand NEP 1 x NEP

Experiments and Validation Details

Entry percentile scores
X-ray source: NSLS BEAMLINE X29A
Spacegroup: P63
Unit cell:
a: 205.088Å b: 205.088Å c: 78.398Å
α: 90° β: 90° γ: 120°
R-values:
R R work R free
0.167 0.166 0.186
Expression system: Homo sapiens

Quick links

Citations

1 review citation

Heparan Sulfate, Mucopolysaccharidosis IIIB and Sulfur Metabolism Disorders.
Kaczor-Kamińska et al. (2022)
3 other articles

6 mentions without citation

Assessment of predicted enzymatic activity of α-N-acetylglucosaminidase variants of unknown significance for CAGI 2016.
Clark et al. (2019)
5 more