› 1now

X-ray diffraction
2.2Å resolution

Human lysosomal beta-hexosaminidase isoform B in complex with (2R,3R,4S,5R)-2-Acetamido-3,4-Dihydroxy-5-Hydroxymethyl-Piperidinium Chloride (GalNAc-isofagomine)

Released:
DOI: 10.2210/pdb1now/pdb
PDB entry 1now coloured by chain, front view.
PDB entry 1now coloured by chain, side view.
PDB entry 1now coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease.
Mark BL, Mahuran DJ, Cherney MM, Zhao D, Knapp S, James MN
J Mol Biol 327 1093-109 (2003)
PMID: 12662933

Function and Biology Details

Reaction catalysed: 
Hydrolysis of terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides
Biochemical function:
Biological process:
Cellular component:
Sequence domains:
Structure domains:

Structure analysis Details

Assembly composition:
homo dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-139695 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (2 distinct):
Beta-hexosaminidase subunit beta Chains: A, B
Molecule details ›
Chains: A, B
Length: 507 amino acids
Theoretical weight: 58.18 KDa
Source organism: Homo sapiens
UniProt:
  • Canonical: P07686 (Residues: 50-556; Coverage: 99%)
Gene names: HCC7, HEXB
Sequence domains:
Structure domains:

Ligands and Environments

Carbohydrate polymer : NEW Components: NAG
Carbohydrate polymer
3 bound ligands:
Ligand IFG 2 x IFG
Ligand GOL 3 x GOL
Ligand SO4 1 x SO4
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 14-BM-C
Spacegroup: P6122
Unit cell:
a: 112.391Å b: 112.391Å c: 397.309Å
α: 90° β: 90° γ: 120°
R-values:
R R work R free
0.194 0.193 0.218

Quick links

Citations

11 review citations

The cell biology of lysosomal storage disorders.
Futerman et al. (2004)
10 more

16 mentions without citation

Crystal structure of human beta-hexosaminidase B: understanding the molecular basis of Sandhoff and Tay-Sachs disease.
Mark et al. (2003)
15 more