› 4fdi

X-ray diffraction
2.2Å resolution

The molecular basis of mucopolysaccharidosis IV A

Released:
DOI: 10.2210/pdb4fdi/pdb
PDB entry 4fdi coloured by chain, front view.
PDB entry 4fdi coloured by chain, side view.
PDB entry 4fdi coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
The structure of human GALNS reveals the molecular basis for mucopolysaccharidosis IV A.
Rivera-Colón Y, Schutsky EK, Kita AZ, Garman SC
J Mol Biol 423 736-51 (2012)
PMID: 22940367

Function and Biology Details

Reaction catalysed: 
Hydrolysis of the 6-sulfate groups of the N-acetyl-D-galactosamine 6-sulfate units of chondroitin sulfate and of the D-galactose 6-sulfate units of keratan sulfate
Biochemical function:
Biological process:
  • not assigned
Cellular component:
Sequence domains:

Structure analysis Details

Assembly composition:
homo dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-152676 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
N-acetylgalactosamine-6-sulfatase Chains: A, B
Molecule details ›
Chains: A, B
Length: 502 amino acids
Theoretical weight: 56.3 KDa
Source organism: Homo sapiens
Expression system: Trichoplusia ni
UniProt:
  • Canonical: P34059 (Residues: 27-522; Coverage: 100%)
Gene name: GALNS
Sequence domains:
Structure domains:

Ligands and Environments

4 bound ligands:
Ligand NAG 4 x NAG
Ligand CA 2 x CA
Ligand CIT 2 x CIT
Ligand GOL 2 x GOL
1 modified residue:
Ligand DDZ 2 x DDZ

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 24-ID-C
Spacegroup: P21
Unit cell:
a: 61.372Å b: 155.522Å c: 62.555Å
α: 90° β: 113.76° γ: 90°
R-values:
R R work R free
0.166 0.164 0.211
Expression system: Trichoplusia ni

Quick links

Citations

13 review citations

Lysosomal enzyme replacement therapies: Historical development, clinical outcomes, and future perspectives.
Solomon et al. (2017)
12 more

15 mentions without citation

Matching the Diversity of Sulfated Biomolecules: Creation of a Classification Database for Sulfatases Reflecting Their Substrate Specificity.
Barbeyron et al. (2016)
14 more