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

X-ray diffraction
1.5Å resolution
PDB entry 1i3n coloured by chain, front view.
PDB entry 1i3n coloured by chain, side view.
PDB entry 1i3n coloured by chain, top view.
Source organism: Homo sapiens
Assembly composition:
homo dimer (preferred)
Entry contents: 1 distinct polypeptide molecule

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Assemblies

Assembly 1 (preferred)
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Assembly Name: UDP-glucose 4-epimerase
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Multimeric state: homo dimer
Accessible surface area: 25861.79 Å2
Buried surface area: 7269.87 Å2
Dissociation area: 1,018.57 Å2
Dissociation energy (ΔGdiss): 11.47 kcal/mol
Dissociation entropy (TΔSdiss): 13.71 kcal/mol
Symmetry number: 2
PDBe Complex ID: PDB-CPX-171995

Macromolecules

UDP-glucose 4-epimerase
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
Pfam: GDP-mannose 4,6 dehydratase
InterPro:
CATH:
SCOP: Tyrosine-dependent oxidoreductases
UDP-glucose 4-epimerase in PDB entry 1i3n, assembly 1, front view.
UDP-glucose 4-epimerase in PDB entry 1i3n, assembly 1, side view.
UDP-glucose 4-epimerase in PDB entry 1i3n, assembly 1, top view.
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