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Ligands
Code Name Style Show Link
CL Chloride ion
EDO 1,2-ethanediol
MG Magnesium ion
NAD Nicotinamide-adenine-dinucleotide
UPG Uridine-5'-diphosphate-glucose
Non-standard Residues
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Code : 1I3K   PDBj   RCSB PDB   PDBe
Header : ISOMERASE
Title : MOLECULAR BASIS FOR SEVERE EPIMERASE-DEFICIENCY GALACTOSEMIA: X-RAY STRUCTURE OF THE HUMAN V94M-SUBSTITUTED UDP-GALACTOSE 4-EPIMERASE
Release Data : 2001-06-20
Compound :
mol_id molecule chains synonym
1 UDP-GLUCOSE 4-EPIMERASE A,B UDP-GALACTOSE 4-EPIMERASE
ec: 5.1.3.2
mutation: V94M
Source :
mol_id organism_scientific organism_common expression_system
1 Homo sapiens  (taxid:9606) Human Pichia pastoris  (taxid:4922)
gene: HGALE
expression_system_vector_type: PLASMID
expression_system_plasmid: PPIC3.5KHGALE
Authors : Thoden, J.B., Wohlers, T.M., Fridovich-Keil, J.L., Holden, H.M.
Keywords : Galactosemia epimerase short-chain dehydrogenase, ISOMERASE
Exp. method : X-RAY DIFFRACTION ( 1.50 Å )
Citation :

Molecular basis for severe epimerase deficiency galactosemia. X-ray structure of the human V94m-substituted UDP-galactose 4-epimerase.

Thoden, J.B.,Wohlers, T.M.,Fridovich-Keil, J.L.  et al.
(2001)  J.Biol.Chem.  276 : 20617 - 20623

PubMed: 11279193
DOI: 10.1074/jbc.M101304200

Chain : A, B
UniProt : Q14376 (GALE_HUMAN)
Reaction: EC: Evidence:
Physiological Direction:
UDP-alpha-D-glucose = UDP-alpha-D-galactose 5.1.3.2 PubMed:22654673
-
UDP-N-acetyl-alpha-D-glucosamine = UDP-N-acetyl-alpha-D- galactosamine 5.1.3.7 PubMed:22654673
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