UDP-galactose 4-epimerase

Function

UDP-galactose 4-epimerase (GalE) reversibly converts UDP-galactose to UDP-glucose. GalE catalyzes the last step in galactose metabolism^[1]. GalE uses NAD+ as a cofactor.

Disease

Mutations in GalE are the cause of galactosemia which is autosomal recessive disorder^[2].

Structural highlights

GalE active site contains the (UDP-gal) and the . Water molecules are shown as red spheres. which produces the ketose intermediate^[3].

Structure of UDP-galactose 4 epimerase complex with NAD and UDP-galactose (PDB entry 2cnb)

Drag the structure with the mouse to rotate

3D structures of UDP-galactose 4 epimerase3D structures of UDP-galactose 4 epimerase

UDP-galactose 4-epimerase 3D structures

ReferencesReferences

↑ Mozzi F, Savoy de Giori G, Font de Valdez G. UDP-galactose 4-epimerase: a key enzyme in exopolysaccharide formation by Lactobacillus casei CRL 87 in controlled pH batch cultures. J Appl Microbiol. 2003;94(2):175-83. PMID:12534808
↑ Park HD, Park KU, Kim JQ, Shin CH, Yang SW, Lee DH, Song YH, Song J. The molecular basis of UDP-galactose-4-epimerase (GALE) deficiency galactosemia in Korean patients. Genet Med. 2005 Nov-Dec;7(9):646-9. PMID:16301867 doi:10.109701.gim.0000194023.27802.2d
↑ Alphey MS, Burton A, Urbaniak MD, Boons GJ, Ferguson MA, Hunter WN. Trypanosoma brucei UDP-galactose-4'-epimerase in ternary complex with NAD+ and the substrate analogue UDP-4-deoxy-4-fluoro-alpha-D-galactose. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2006 Sep 1;62(Pt, 9):829-34. Epub 2006 Aug 11. PMID:16946458 doi:10.1107/S1744309106028740

</StructureSection>

Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

Michal Harel, Alexander Berchansky

[1] Mozzi F, Savoy de Giori G, Font de Valdez G. UDP-galactose 4-epimerase: a key enzyme in exopolysaccharide formation by Lactobacillus casei CRL 87 in controlled pH batch cultures. J Appl Microbiol. 2003;94(2):175-83. PMID:12534808

[2] Park HD, Park KU, Kim JQ, Shin CH, Yang SW, Lee DH, Song YH, Song J. The molecular basis of UDP-galactose-4-epimerase (GALE) deficiency galactosemia in Korean patients. Genet Med. 2005 Nov-Dec;7(9):646-9. PMID:16301867 doi:10.109701.gim.0000194023.27802.2d

[3] Alphey MS, Burton A, Urbaniak MD, Boons GJ, Ferguson MA, Hunter WN. Trypanosoma brucei UDP-galactose-4'-epimerase in ternary complex with NAD+ and the substrate analogue UDP-4-deoxy-4-fluoro-alpha-D-galactose. Acta Crystallogr Sect F Struct Biol Cryst Commun. 2006 Sep 1;62(Pt, 9):829-34. Epub 2006 Aug 11. PMID:16946458 doi:10.1107/S1744309106028740

[1]

[2]

[3]