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1I3L

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

Entity
Entity IDChain IDDescriptionTypeChain lengthFormula weightNumber of moleculesDB Name (Accession)Biological sourceDescriptive keywords
1A, B
(A, B)
UDP-GLUCOSE 4-EPIMERASEpolymer34838384.72UniProt (Q14376)
Pfam (PF16363)
Homo sapiens (human)UDP-GALACTOSE 4-EPIMERASE
2C, H
(A, B)
GALACTOSE-URIDINE-5'-DIPHOSPHATEnon-polymer566.32Chemie (GDU)
3D, I, J
(A, B)
CHLORIDE IONnon-polymer35.53Chemie (CL)
4E
(A)
MAGNESIUM IONnon-polymer24.31Chemie (MG)
5F, K
(A, B)
NICOTINAMIDE-ADENINE-DINUCLEOTIDEnon-polymer663.42Chemie (NAD)
6G, L
(A, B)
1,2-ETHANEDIOLnon-polymer62.12Chemie (EDO)
7M, N
(A, B)
waterwater18.0935Chemie (HOH)
Sequence modifications
A, B: 1 - 348 (UniProt: Q14376)
PDBExternal DatabaseDetails
Met 94Val 94engineered mutation
Sequence viewer
Contents of the asymmetric unit
PolymersNumber of chains2
Total formula weight76769.5
Non-Polymers*Number of molecules10
Total formula weight2714.3
All*Total formula weight79483.7
*Water molecules are not included.

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PDB entries from 2025-06-18

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