› 7qqg

X-ray diffraction
2.43Å resolution

Crystal structure of MYORG bound to 1-deoxygalactonojirimycin

Released:
DOI: 10.2210/pdb7qqg/pdb
PDB entry 7qqg coloured by chain, front view.
PDB entry 7qqg coloured by chain, side view.
PDB entry 7qqg coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
The primary familial brain calcification-associated protein MYORG is an α-galactosidase with restricted substrate specificity.
Meek RW, Brockerman J, Fordwour OB, Zandberg WF, Davies GJ, Vocadlo DJ
PLoS Biol 20 e3001764 (2022)
PMID: 36129849

Function and Biology Details

Biochemical function:
Biological process:
Cellular component:
  • not assigned
Sequence domains:

Structure analysis Details

Assembly composition:
homo dimer (preferred)
Assembly name:
PDBe Complex ID:
PDB-CPX-180066 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecules (3 distinct):
Myogenesis-regulating glycosidase Chains: A, B, C, D
Molecule details ›
Chains: A, B, C, D
Length: 636 amino acids
Theoretical weight: 72.46 KDa
Source organism: Homo sapiens
Expression system: Trichoplusia ni
UniProt:
  • Canonical: Q6NSJ0 (Residues: 80-714; Coverage: 89%)
Gene names: KIAA1161, MYORG
Sequence domains:

Ligands and Environments

Carbohydrate polymer : NEW Components: NAG
Carbohydrate polymer
Carbohydrate polymer : NEW Components: NAG, FUC
Carbohydrate polymer
3 bound ligands:
Ligand NAG 10 x NAG
Ligand DGJ 4 x DGJ
Ligand MLI 2 x MLI
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: DIAMOND BEAMLINE I04
Spacegroup: P1
Unit cell:
a: 73.113Å b: 78.882Å c: 176.221Å
α: 80.777° β: 80.209° γ: 62.641°
R-values:
R R work R free
0.225 0.224 0.25
Expression system: Trichoplusia ni

Quick links

Citations

2 review citations

Fahr's disease linked to a novel mutation in MYORG variants manifesting as paroxysmal limb stiffness and dysarthria: Case report and literature review.
Zhao et al. (2023)
1 more