› 1e33

X-ray diffraction
2.5Å resolution

Crystal structure of an Arylsulfatase A mutant P426L

Released:
DOI: 10.2210/pdb1e33/pdb
PDB entry 1e33 coloured by chain, front view.
PDB entry 1e33 coloured by chain, side view.
PDB entry 1e33 coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Defective oligomerization of arylsulfatase a as a cause of its instability in lysosomes and metachromatic leukodystrophy.
von Bülow R, Schmidt B, Dierks T, Schwabauer N, Schilling K, Weber E, Usón I, von Figura K
J Biol Chem 277 9455-61 (2002)
PMID: 11777924

Function and Biology Details

Reaction catalysed: 
A cerebroside 3-sulfate + H(2)O = a cerebroside + sulfate
Biochemical function:
Biological process:
Cellular component:
Sequence domains:
Structure domain:

Structure analysis Details

Assembly composition:
homo dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-147294 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (2 distinct):
Arylsulfatase A Chain: P
Molecule details ›
Chain: P
Length: 489 amino acids
Theoretical weight: 51.98 KDa
Source organism: Homo sapiens
Expression system: Mus musculus
UniProt:
  • Canonical: P15289 (Residues: 19-507; Coverage: 100%)
Gene name: ARSA
Sequence domains:
Structure domains:

Ligands and Environments

Carbohydrate polymer : NEW Components: NAG
Carbohydrate polymer
1 bound ligand:
Ligand MG 1 x MG
1 modified residue:
Ligand DDZ 1 x DDZ

Experiments and Validation Details

Entry percentile scores
X-ray source: EMBL/DESY, HAMBURG BEAMLINE X11
Spacegroup: I422
Unit cell:
a: 131.4Å b: 131.4Å c: 192Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.187 0.185 0.25
Expression system: Mus musculus

Quick links

Citations

6 review citations

Mutation Update of ARSA and PSAP Genes Causing Metachromatic Leukodystrophy.
Cesani et al. (2016)
5 more

1 mention without citation

Purification, Characterization, and Structural Studies of a Sulfatase from Pedobacter yulinensis.
Schlachter et al. (2021)