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1I3N

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, D, H, I
(A, B)
CHLORIDE IONnon-polymer35.54Chemie (CL)
3E
(A)
MAGNESIUM IONnon-polymer24.31Chemie (MG)
4F, J
(A, B)
NICOTINAMIDE-ADENINE-DINUCLEOTIDEnon-polymer663.42Chemie (NAD)
5G, K
(A, B)
URIDINE-DIPHOSPHATE-N-ACETYLGLUCOSAMINEnon-polymer607.42Chemie (UD1)
6L, M
(A, B)
waterwater18.0925Chemie (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 molecules9
Total formula weight2707.7
All*Total formula weight79477.1
*Water molecules are not included.

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PDB entries from 2024-10-30

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