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

Source organism: Homo sapiens
Biochemical function: protein homodimerization activity
Biological process: galactose catabolic process via UDP-galactose
Cellular component: cytosol

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EC 5.1.3.7: UDP-N-acetylglucosamine 4-epimerase

Reaction catalysed:
UDP-N-acetyl-alpha-D-glucosamine = UDP-N-acetyl-alpha-D-galactosamine
Systematic name:
UDP-N-acetyl-alpha-D-glucosamine 4-epimerase
Alternative Name(s):
  • UDP acetylglucosamine epimerase
  • UDP-GlcNAc 4-epimerase
  • UDP-N-acetyl-D-glucosamine 4-epimerase
  • Uridine 5'-diphospho-N-acetylglucosamine-4-epimerase
  • Uridine diphosphate N-acetylglucosamine-4-epimerase
  • Uridine diphosphoacetylglucosamine epimerase

EC 5.1.3.2: UDP-glucose 4-epimerase

Reaction catalysed:
UDP-alpha-D-glucose = UDP-alpha-D-galactose
Systematic name:
UDP-alpha-D-glucose 4-epimerase
Alternative Name(s):
  • 4-epimerase
  • Galactowaldenase
  • UDP-D-galactose 4-epimerase
  • UDP-galactose 4-epimerase
  • UDP-glucose epimerase
  • UDPG-4-epimerase
  • UDPgalactose 4-epimerase
  • UDPglucose 4-epimerase
  • Uridine diphosphate galactose 4-epimerase
  • Uridine diphosphate glucose 4-epimerase
  • Uridine diphospho-galactose-4-epimerase
  • Uridine diphosphoglucose 4-epimerase
  • Uridine diphosphoglucose epimerase

GO terms

Sequence family

Pfam Protein family (Pfam)
PF16363
Domain description: GDP-mannose 4,6 dehydratase
Occurring in:
  1. UDP-glucose 4-epimerase
The deposited structure of PDB entry 1i3n contains 2 copies of Pfam domain PF16363 (GDP-mannose 4,6 dehydratase) in UDP-glucose 4-epimerase. Showing 1 copy in chain A.

InterPro InterPro annotations
IPR005886
Domain description: UDP-glucose 4-epimerase
Occurring in:
  1. UDP-glucose 4-epimerase
IPR016040
Domain description: NAD(P)-binding domain
Occurring in:
  1. UDP-glucose 4-epimerase
IPR036291
Domain description: NAD(P)-binding domain superfamily
Occurring in:
  1. UDP-glucose 4-epimerase

Structure domains

CATH CATH domains
3.90.25.10
Class: Alpha Beta
Architecture: Alpha-Beta Complex
Topology: UDP-galactose 4-epimerase; domain 1
Homology: UDP-galactose 4-epimerase, domain 1
Occurring in:
  1. UDP-glucose 4-epimerase
The deposited structure of PDB entry 1i3n contains 2 copies of CATH domain 3.90.25.10 (UDP-galactose 4-epimerase; domain 1) in UDP-glucose 4-epimerase. Showing 1 copy in chain A.
3.40.50.720
Class: Alpha Beta
Architecture: 3-Layer(aba) Sandwich
Topology: Rossmann fold
Homology: NAD(P)-binding Rossmann-like Domain
Occurring in:
  1. UDP-glucose 4-epimerase
The deposited structure of PDB entry 1i3n contains 2 copies of CATH domain 3.40.50.720 (Rossmann fold) in UDP-glucose 4-epimerase. Showing 1 copy in chain A.
SCOP SCOP annotation
51751
Class: Alpha and beta proteins (a/b)
Fold: NAD(P)-binding Rossmann-fold domains
Superfamily: NAD(P)-binding Rossmann-fold domains
Occurring in:
  1. UDP-glucose 4-epimerase
The deposited structure of PDB entry 1i3n contains 2 copies of SCOP domain 51751 (Tyrosine-dependent oxidoreductases) in UDP-glucose 4-epimerase. Showing 1 copy in chain A.

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