› 1i3k

X-ray diffraction
1.5Å resolution

MOLECULAR BASIS FOR SEVERE EPIMERASE-DEFICIENCY GALACTOSEMIA: X-RAY STRUCTURE OF THE HUMAN V94M-SUBSTITUTED UDP-GALACTOSE 4-EPIMERASE

Released:
DOI: 10.2210/pdb1i3k/pdb
PDB entry 1i3k coloured by chain, front view.
PDB entry 1i3k coloured by chain, side view.
PDB entry 1i3k coloured by chain, top view.
Source organism: Homo sapiens
Primary publication:
Molecular basis for severe epimerase deficiency galactosemia. X-ray structure of the human V94m-substituted UDP-galactose 4-epimerase.
Thoden JB, Wohlers TM, Fridovich-Keil JL, Holden HM
J Biol Chem 276 20617-23 (2001)
PMID: 11279193

Function and Biology Details

Reactions catalysed: 
UDP-alpha-D-glucose = UDP-alpha-D-galactose
UDP-N-acetyl-alpha-D-glucosamine = UDP-N-acetyl-alpha-D-galactosamine
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-171995 (preferred)
Entry contents:
1 distinct polypeptide molecule
Macromolecule:
UDP-glucose 4-epimerase Chains: A, B
Molecule details ›
Chains: A, B
Length: 348 amino acids
Theoretical weight: 38.38 KDa
Source organism: Homo sapiens
Expression system: Komagataella pastoris
UniProt:
  • Canonical: Q14376 (Residues: 1-348; Coverage: 100%)
Gene name: GALE
Sequence domains: GDP-mannose 4,6 dehydratase
Structure domains:

Ligands and Environments


Cofactor: Ligand NAD 2 x NAD
4 bound ligands:
Ligand CL 4 x CL
Ligand MG 1 x MG
Ligand UPG 2 x UPG
Ligand EDO 3 x EDO
No modified residues

Experiments and Validation Details

Entry percentile scores
X-ray source: APS BEAMLINE 19-BM
Spacegroup: P212121
Unit cell:
a: 78.1Å b: 89.9Å c: 96.9Å
α: 90° β: 90° γ: 90°
R-values:
R R work R free
0.175 0.174 0.198
Expression system: Komagataella pastoris

Quick links

Citations

1 review citation

Medium- and short-chain dehydrogenase/reductase gene and protein families : the SDR superfamily: functional and structural diversity within a family of metabolic and regulatory enzymes.
Kavanagh et al. (2008)
25 other articles