1I3N | Isomerase | date | Feb 15, 2001 | ||||||||||||
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title | Molecular Basis For Severe Epimerase-Deficiency Galactosemia: X-Ray Structure Of The Human V94m- Substituted Udp-Galactose 4-Epimerase | ||||||||||||||
authors | J.B.Thoden, T.M.Wohlers, J.L.Fridovich-Keil, H.M.Holden | ||||||||||||||
compound | source | ||||||||||||||
Molecule: Udp-Glucose 4-Epimerase Chain: A, B Synonym: Udp-Galactose 4-Epimerase Ec: 5.1.3.2 Engineered: Yes Mutation: Yes |
Organism_scientific: Homo Sapiens Organism_common: Human Gene: Hgale Expression_system: Pichia Pastoris Expression_system_common: Yeast Expression_system_strain: Gs115 Expression_system_vector_type: Plasmid Expression_system_plasmid: Ppic3.5khgale | ||||||||||||||
symmetry | Space Group: P 21 21 21 | R_factor | 0.180 | ||||||||||||
crystal cell |
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method | X-Ray Diffraction | resolution | 1.50 Å | ||||||||||||
ligand | CL, MG, NAD, UD1, UD2 | enzyme | UDP-glucose 4-epimerase. UDP-galactose 4-epimerase. Uridine diphosphoglucose epimerase. Galactowaldenase. UDPG-4-epimerase. Uridine diphosphate galactose 4-epimerase. Uridine diphospho-galactose-4-epimerase. UDP-glucose epimerase. 4-epimerase. Uridine diphosphoglucose 4-epimerase. Uridine diphosphate glucose 4-epimerase. UDP-D-galactose 4-epimerase. Isomerase E.C.5.1.3.2 | ||||||||||||
related structures | by homologous chain: 1I3K, 1I3L | ||||||||||||||
similarity | Belongs to the sugar Epimerase family. | ||||||||||||||
catalytic activ. | Udp-glucose = udp-galactose. | ||||||||||||||
pathway | Galactose metabolism; third step. | ||||||||||||||
genes | B0759, GALD, GALE (E. coli); GALE (H. sapiens) | ||||||||||||||
function | Catalyzes two distinct but analogous reactions epimerization of udp-glucose to udp-galactose and the epimerization of udp-n-acetylglucosamine to udp-n- acetylgalactosamine. | ||||||||||||||
Gene Ontology | chain A: GO:0003824, GO:0003978, GO:0005975, GO:0006012, GO:0009225, GO:0016853, GO:0016857 chain B: GO:0003824, GO:0003978, GO:0005975, GO:0006012, GO:0009225, GO:0016853, GO:0016857 | ||||||||||||||
disease | Galactose epimerase deficiency There are two clinically distinct forms of edg. Defects in gale are the cause of epimerase-deficiency galactosemia (edg) [mim. 230350]; also known as galactosemia iii:(1) a benign, or 'peripheral' form with no detectable gale activity in red blood cells:(2) a much rarer and 'generalized' form with undetectable levels of gale activity in all tissues examined. | ||||||||||||||
Primary reference | Molecular basis for severe epimerase deficiency galactosemia. X-ray structure of the human V94m-substituted UDP-galactose 4-epimerase., Thoden JB, Wohlers TM, Fridovich-Keil JL, Holden HM, J Biol Chem 2001 Jun 8;276(23):20617-23. PMID:11279193 |
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Structure-derived information |
- Domain d1i3na_, region A [Jmol] [rasmolscript] [script source] - Domain d1i3nb_, region B [Jmol] [rasmolscript] [script source] |
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