› 8p41

X-ray diffraction
1.83Å resolution

Crystal structure of glucocerebrosidase in complex with allosteric activator

Released:
DOI: 10.2210/pdb8p41/pdb
PDB entry 8p41 coloured by chain, front view.
PDB entry 8p41 coloured by chain, side view.
PDB entry 8p41 coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Identification of ß-Glucocerebrosidase Activators for Glucosylceramide hydrolysis.
Schulze MED, Scholz D, Jnoff E, Hall A, Melin J, Sands ZA, Rodriguez E, Andre VM
ChemMedChem e202300548 (2024)
PMID: 38381042

Function and Biology Details

Reactions catalysed: 
D-glucosyl-N-acylsphingosine + H(2)O = D-glucose + N-acylsphingosine
D-galactosyl-N-acylsphingosine + H(2)O = D-galactose + N-acylsphingosine
Biochemical function:
Biological process:
Cellular component:

Structure analysis Details

Assembly composition:
monomeric (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-137377 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (2 distinct):
Lysosomal acid glucosylceramidase Chains: A, B
Molecule details ›
Chains: A, B
Length: 497 amino acids
Theoretical weight: 55.64 KDa
Source organism: Homo sapiens
Expression system: Cricetulus griseus
UniProt:
  • Canonical: P04062 (Residues: 40-536; Coverage: 100%)
Gene names: GBA, GBA1, GC, GLUC
Sequence domains:

Ligands and Environments

Carbohydrate polymer : NEW Components: NAG
Carbohydrate polymer
3 bound ligands:
Ligand NAG 1 x NAG
Ligand SO4 16 x SO4
Ligand WYC 2 x WYC
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: SOLEIL BEAMLINE PROXIMA 1
Spacegroup: C2221
Unit cell:
a: 109.36Å b: 285.53Å c: 91.72Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.206 0.204 0.238
Expression system: Cricetulus griseus

Quick links

Citations

1 mention without citation

Human mitochondrial chaperone (mtHSP70) and cysteine desulfurase (NFS1) bind preferentially to the disordered conformation, whereas co-chaperone (HSC20) binds to the structured conformation of the iron-sulfur cluster scaffold protein (ISCU).
Cai et al. (2013)