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1I3K

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, I, J
(A, B)
CHLORIDE IONnon-polymer35.54Chemie (CL)
PubChem (312)
3D
(A)
MAGNESIUM IONnon-polymer24.31Chemie (MG)
PubChem (888)
4E, K
(A, B)
NICOTINAMIDE-ADENINE-DINUCLEOTIDEnon-polymer663.42Chemie (NAD)
5F, L
(A, B)
URIDINE-5'-DIPHOSPHATE-GLUCOSEnon-polymer566.32Chemie (UPG)
PubChem (1166)
PubChem (439156)
PubChem (448801)
PubChem (6857540)
PubChem (12133414)
PubChem (13008857)
PubChem (13987142)
PubChem (16667381)
PubChem (20776702)
PubChem (20776703)
PubChem (25011746)
PubChem (45109771)
PubChem (46226441)
PubChem (46936243)
PubChem (16760700)
PubChem (20776705)
PubChem (20776706)
PubChem (23724458)
PubChem (56650783)
PubChem (56846106)
PubChem (8629)
PubChem (18068)
PubChem (439158)
PubChem (448873)
PubChem (640055)
PubChem (92328335)
PubChem (92974127)
PubChem (53477679)
PubChem (6857410)
PubChem (56845790)
PubChem (124933210)
PubChem (129850353)
PubChem (11734460)
PubChem (58538034)
PubChem (155893098)
PubChem (158571248)
PubChem (162920685)
PubChem (168265389)
PubChem (59720988)
PubChem (88869506)
PubChem (88869507)
PubChem (88869743)
PubChem (88870643)
PubChem (88883029)
PubChem (88910951)
PubChem (88941526)
PubChem (89321600)
PubChem (89360378)
PubChem (89847362)
PubChem (90056247)
PubChem (90950359)
PubChem (91746280)
PubChem (92974126)
PubChem (92974128)
PubChem (92974129)
PubChem (93535589)
PubChem (102429624)
PubChem (124933209)
PubChem (134736387)
PubChem (134736388)
PubChem (138911444)
PubChem (162920683)
PubChem (162920684)
PubChem (163626061)
PubChem (166642644)
PubChem (168720052)
PubChem (174027848)
6G, M, N
(A, B)
1,2-ETHANEDIOLnon-polymer62.13Chemie (EDO)
7O, P
(A, B)
waterwater18.0977Chemie (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 molecules12
Total formula weight2811.8
All*Total formula weight79581.2
*Water molecules are not included.

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PDB entries from 2026-04-08

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