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alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
CDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + CDP-galactose
TDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + TDP-galactose
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
UDP-alpha-D-glucose + N-acetyl-alpha-D-galactosamine 1-phosphate
alpha-D-glucose 1-phosphate + UDP-N-acetyl-alpha-D-galactosamine
UDP-alpha-D-glucose + N-acetyl-alpha-D-glucosamine 1-phosphate
alpha-D-glucose 1-phosphate + UDP-N-acetyl-alpha-D-glucosamine
UDP-alphaS-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alphaS-galactose
UDP-glucose + 2-methylimidazole
uridine 5'-phospho-2-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 4-methylimidazole
uridine 5'-phospho-4-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
UDP-glucose + D-galactose 1-phosphate
D-glucose 1-phosphate + UDP-galactose
-
-
-
-
?
UDP-glucose + imidazole
uridine 5'-phosphoimidazole + alpha-D-glucose 1-phosphate
uridine 5'-phospho-2,4,5-trimethylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 2,4,5-trimethylimidazole
uridine 5'-phospho-2-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 2-methylimidazole
uridine 5'-phospho-3-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 3-methylimidazole
uridine 5'-phospho-4-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 4-methylimidazole
uridine 5'-phospho-5-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 5-methylimidazole
UTP + alpha-D-galactose 1-phosphate
diphosphate + UDP-alpha-D-galactose
-
-
-
-
?
additional information
?
-
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose

UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
-
-
-
-
r
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
-
-
-
r
CDP-glucose + alpha-D-galactose 1-phosphate

alpha-D-glucose 1-phosphate + CDP-galactose
-
no activity
-
-
?
CDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + CDP-galactose
-
low activity
-
-
?
TDP-glucose + alpha-D-galactose 1-phosphate

alpha-D-glucose 1-phosphate + TDP-galactose
-
reaction at 60% the rate of UDP-glucose
-
-
r
TDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + TDP-galactose
-
8.7% activity compared to UDP-glucose
-
-
r
TDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + TDP-galactose
-
no activity
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate

alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
the enzyme is involved in galactose metabolism via the Leloir pathway
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
binding of the glucose moiety of the substrate, but not the UDP moiety, gives rise to a large structural change around the active site
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
the enzyme is involved in galactose metabolism via the Leloir pathway
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
binding of the glucose moiety of the substrate, but not the UDP moiety, gives rise to a large structural change around the active site
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
r
UDP-alpha-D-glucose + N-acetyl-alpha-D-galactosamine 1-phosphate

alpha-D-glucose 1-phosphate + UDP-N-acetyl-alpha-D-galactosamine
-
-
-
?
UDP-alpha-D-glucose + N-acetyl-alpha-D-galactosamine 1-phosphate
alpha-D-glucose 1-phosphate + UDP-N-acetyl-alpha-D-galactosamine
-
-
-
?
UDP-alpha-D-glucose + N-acetyl-alpha-D-glucosamine 1-phosphate

alpha-D-glucose 1-phosphate + UDP-N-acetyl-alpha-D-glucosamine
-
-
-
?
UDP-alpha-D-glucose + N-acetyl-alpha-D-glucosamine 1-phosphate
alpha-D-glucose 1-phosphate + UDP-N-acetyl-alpha-D-glucosamine
-
-
-
?
UDP-alphaS-glucose + alpha-D-galactose 1-phosphate

alpha-D-glucose 1-phosphate + UDP-alphaS-galactose
-
stereochemistry: enzyme accepts (Rp)-UDP-alphaS-glucose converting it to (Rp)-UDP-alphaS-galactose, the overall retention of configuration arises from inversion in each of the two steps
(Rp1)-UDP-alphaS-galactose, i.e. uridine 5'-(1-thiodiphosphate) galactose
r
UDP-alphaS-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alphaS-galactose
-
i.e. uridine 5'-(1-thiodiphosphate) glucose
(Rp1)-UDP-alphaS-galactose, i.e. uridine 5'-(1-thiodiphosphate) galactose
r
UDP-alphaS-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alphaS-galactose
-
(Rp1)-UDP-alphaS-glucose
(Rp1)-UDP-alphaS-galactose, i.e. uridine 5'-(1-thiodiphosphate) galactose
r
UDP-glucose + 2-methylimidazole

uridine 5'-phospho-2-methylimidazole + alpha-D-glucose 1-phosphate
-
kinetic study
-
r
UDP-glucose + 2-methylimidazole
uridine 5'-phospho-2-methylimidazole + alpha-D-glucose 1-phosphate
-
mutant H166G, i.e. UDP-hexose synthase
-
r
UDP-glucose + 4-methylimidazole

uridine 5'-phospho-4-methylimidazole + alpha-D-glucose 1-phosphate
-
kinetic study
-
r
UDP-glucose + 4-methylimidazole
uridine 5'-phospho-4-methylimidazole + alpha-D-glucose 1-phosphate
-
mutant H166G, i.e. UDP-hexose synthase
-
r
UDP-glucose + alpha-D-galactose 1-phosphate

alpha-D-glucose 1-phosphate + UDP-galactose
-
highly specific for the substrates
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
involved in conversion of galactose into alpha-D-glucose 1-phosphate, Leloir pathway
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
important step in galactose metabolism
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
central enzyme in the classical Lelior pathway, but not in galactose metabolsim
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
stereochemistry: enzyme accepts (Rp)-UDP-alphaS-glucose converting it to (Rp)-UDP-alphaS-galactose, the overall retention of configuration arises from inversion in each of the two steps
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
formation of a covalent uridylyl-enzyme intermediate, i.e. UMP-enzyme
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
formation of a covalent uridylyl-enzyme intermediate, i.e. UMP-enzyme
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
formation of a covalent uridylyl-enzyme intermediate, i.e. UMP-enzyme
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
highly specific for the substrates
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
recombinant bifunctional chimeric fusion protein
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
2nd step of Leloir pathway
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
involved in conversion of galactose into alpha-D-glucose 1-phosphate, Leloir pathway
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
highly specific for the substrates
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
involved in conversion of galactose into alpha-D-glucose 1-phosphate, Leloir pathway
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
2-step mechanism
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
galactosemia with altered activity of the follicle-stimulating hormone FSH
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
important step in galactose metabolism
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
-
?
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
2nd step of Leloir pathway
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
2nd step of Leloir pathway
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
involved in the biosynthesis of follicle-stimulating hormone FSH
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
transcriptional regulation is complex and not directly induced by substrate levels
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
2nd step of Leloir pathway
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + imidazole

uridine 5'-phosphoimidazole + alpha-D-glucose 1-phosphate
-
wild-type, 800fold reduced activity compared to normal reaction intermediate uridylyl-enzyme as substrate, pH-dependent
-
r
UDP-glucose + imidazole
uridine 5'-phosphoimidazole + alpha-D-glucose 1-phosphate
-
mutant H166A
-
r
UDP-glucose + imidazole
uridine 5'-phosphoimidazole + alpha-D-glucose 1-phosphate
-
mutant H166G, i.e. UDP-hexose synthase
-
r
UDP-glucose + imidazole
uridine 5'-phosphoimidazole + alpha-D-glucose 1-phosphate
-
mutant H166G, i.e. UDP-hexose synthase
-
r
UDP-glucose + imidazole
uridine 5'-phosphoimidazole + alpha-D-glucose 1-phosphate
-
equilibrium study and constants
-
r
UDP-glucose + imidazole
uridine 5'-phosphoimidazole + alpha-D-glucose 1-phosphate
-
equilibrium study and constants
-
r
uridine 5'-phospho-2,4,5-trimethylimidazole + alpha-D-glucose 1-phosphate

UDP-glucose + 2,4,5-trimethylimidazole
-
kinetic study
-
r
uridine 5'-phospho-2,4,5-trimethylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 2,4,5-trimethylimidazole
-
mutant H166G, i.e. UDP-hexose synthase
-
r
uridine 5'-phospho-2-methylimidazole + alpha-D-glucose 1-phosphate

UDP-glucose + 2-methylimidazole
-
kinetic study
-
r
uridine 5'-phospho-2-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 2-methylimidazole
-
mutant H166G, i.e. UDP-hexose synthase
-
r
uridine 5'-phospho-3-methylimidazole + alpha-D-glucose 1-phosphate

UDP-glucose + 3-methylimidazole
-
kinetic study
-
r
uridine 5'-phospho-3-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 3-methylimidazole
-
mutant H166G, i.e. UDP-hexose synthase
-
r
uridine 5'-phospho-4-methylimidazole + alpha-D-glucose 1-phosphate

UDP-glucose + 4-methylimidazole
-
kinetic study
-
r
uridine 5'-phospho-4-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 4-methylimidazole
-
mutant H166G, i.e. UDP-hexose synthase
-
r
uridine 5'-phospho-5-methylimidazole + alpha-D-glucose 1-phosphate

UDP-glucose + 5-methylimidazole
-
kinetic study
-
r
uridine 5'-phospho-5-methylimidazole + alpha-D-glucose 1-phosphate
UDP-glucose + 5-methylimidazole
-
mutant H166G, i.e. UDP-hexose synthase
-
r
additional information

?
-
-
no activity with CDP-glucose
-
-
?
additional information
?
-
-
no activity with ADP-glucose or GDP-glucose
-
-
?
additional information
?
-
no substrates: N-acetyl-alpha-D-galactose 1-phosphate, N-acetyl-alpha-D-glucose 1-phosphate
-
-
?
additional information
?
-
no substrates: N-acetyl-alpha-D-galactose 1-phosphate, N-acetyl-alpha-D-glucose 1-phosphate
-
-
?
additional information
?
-
-
no substrates: N-acetyl-alpha-D-galactose 1-phosphate, N-acetyl-alpha-D-glucose 1-phosphate
-
-
?
additional information
?
-
no substrates: N-acetyl-alpha-D-galactose 1-phosphate, N-acetyl-alpha-D-glucose 1-phosphate
-
-
?
additional information
?
-
no substrates: N-acetyl-alpha-D-galactose 1-phosphate, N-acetyl-alpha-D-glucose 1-phosphate
-
-
?
additional information
?
-
-
no activity with fructose 1-phosphate and galactose 6-phosphate
-
-
?
additional information
?
-
-
no activity with ADP-glucose or GDP-glucose
-
-
?
additional information
?
-
-
no activity with xylose 1-phosphate, and IDP-glucose
-
-
?
additional information
?
-
-
no activity with fructose 1-phosphate and galactose 6-phosphate
-
-
?
additional information
?
-
-
no activity with ADP-glucose or GDP-glucose
-
-
?
additional information
?
-
-
no activity with ribose 1-phosphate and mannose 6-phosphate
-
-
?
additional information
?
-
-
the enzyme has a strict requirement for UDP-glucose or UDP-galactose as substrates, while GDP-glucose, ADP-glucose, TDP-glucose, CDP-glucose, UDPxylose, and UDP-mannose are all unable to support activity
-
-
?
additional information
?
-
-
no activity with UDP-xylose, ADP-glucose, and galactose 6-phosphate
-
-
?
additional information
?
-
-
-
-
-
?
additional information
?
-
-
inverse relationship between enzyme activity and galactose sensitivity
-
-
?
additional information
?
-
-
the enzyme has a strict requirement for UDP-glucose or UDP-galactose as substrates, while GDP-glucose, ADP-glucose, TDP-glucose, CDP-glucose, UDPxylose, and UDP-mannose are all unable to support activity
-
-
?
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UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
UTP + alpha-D-galactose 1-phosphate
diphosphate + UDP-alpha-D-galactose
-
-
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate

alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
r
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
the enzyme is involved in galactose metabolism via the Leloir pathway
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
the enzyme is involved in galactose metabolism via the Leloir pathway
-
-
?
UDP-alpha-D-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-alpha-D-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate

alpha-D-glucose 1-phosphate + UDP-galactose
-
involved in conversion of galactose into alpha-D-glucose 1-phosphate, Leloir pathway
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
important step in galactose metabolism
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
central enzyme in the classical Lelior pathway, but not in galactose metabolsim
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
2nd step of Leloir pathway
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
involved in conversion of galactose into alpha-D-glucose 1-phosphate, Leloir pathway
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
involved in conversion of galactose into alpha-D-glucose 1-phosphate, Leloir pathway
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
galactosemia with altered activity of the follicle-stimulating hormone FSH
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
important step in galactose metabolism
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
-
?
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
essential enzyme in galactose metabolism, deficiency causes genetic disorder galactosemia, e.g. mutant Duarte variant
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
2nd step of Leloir pathway
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
2nd step of Leloir pathway
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
involved in the biosynthesis of follicle-stimulating hormone FSH
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
transcriptional regulation is complex and not directly induced by substrate levels
-
-
r
UDP-glucose + alpha-D-galactose 1-phosphate
alpha-D-glucose 1-phosphate + UDP-galactose
-
2nd step of Leloir pathway
-
r
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
Please wait a moment until the data is sorted. This message will disappear when the data is sorted.
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Carcinoma, Ovarian Epithelial
Galactose-1-phosphate uridyl transferase (GALT) genotype and phenotype, galactose consumption, and the risk of borderline and invasive ovarian cancer (United States).
Cataract
Association of presenile cataract with galactose-1-phosphate uridyl transferase gene mutations.
Cataract
Clinical features of galactokinase deficiency: a review of the literature.
Cataract
Negative screening tests in classical galactosaemia caused by S135L homozygosity.
Cataract
[Activity of enzymes of galactose metabolism in so-called congenital cataract (author's transl)]
Congenital Disorders of Glycosylation
Hypoglycosylation with increased fucosylation and branching of serum transferrin N-glycans in untreated galactosemia.
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Hypoglycosylation with increased fucosylation and branching of serum transferrin N-glycans in untreated galactosemia.
Craniosynostoses
A Novel Large Deletion Encompassing the Whole of the Galactose-1-Phosphate Uridyltransferase (GALT) Gene and Extending into the Adjacent Interleukin 11 Receptor Alpha (IL11RA) Gene Causes Classic Galactosemia Associated with Additional Phenotypic Abnormalities.
Endometriosis
Mutation of galactose-1-phosphate uridyl transferase and its association with ovarian cancer and endometriosis.
Fanconi Syndrome
Defective galactose oxidation in a patient with glycogen storage disease and Fanconi syndrome.
galactokinase deficiency
Biokinetics of galactose in the homozygotes and heterozygotes of both forms of galactosemia.
galactokinase deficiency
Clinical features of galactokinase deficiency: a review of the literature.
galactokinase deficiency
Hemoglobin A1 in galactosemia, a possible role in monitoring dietary compliance.
galactokinase deficiency
Recommendations for newborn screening for galactokinase deficiency: A systematic review and evaluation of Dutch newborn screening data.
galactokinase deficiency
Simultaneous quantitative estimation of galactose-1-phosphate and galactose in blood for the diagnosis of galactosemia.
Galactosemias
A Case of Classical Galactosemia: Identification and Characterization of 3 Distinct Mutations in Galactose-1-Phosphate Uridyl Transferase (GALT) Gene in a Single Family.
Galactosemias
A De Novo Variant in Galactose-1-P Uridylyltransferase (GALT) Leading to Classic Galactosemia.
Galactosemias
A Novel Large Deletion Encompassing the Whole of the Galactose-1-Phosphate Uridyltransferase (GALT) Gene and Extending into the Adjacent Interleukin 11 Receptor Alpha (IL11RA) Gene Causes Classic Galactosemia Associated with Additional Phenotypic Abnormalities.
Galactosemias
Abnormal N-glycan fucosylation, galactosylation, and sialylation of IgG in adults with classical galactosemia, influence of dietary galactose intake.
Galactosemias
An extensive computational approach to analyze and characterize the functional mutations in the galactose-1-phosphate uridyl transferase (GALT) protein responsible for classical galactosemia.
Galactosemias
Analysis of common mutations in the galactose-1-phosphate uridyl transferase gene: new assays to increase the sensitivity and specificity of newborn screening for galactosemia.
Galactosemias
Assessment of galactose-1-phosphate uridyltransferase activity in cells and tissues.
Galactosemias
Biokinetics of galactose in the homozygotes and heterozygotes of both forms of galactosemia.
Galactosemias
Clinical features of galactokinase deficiency: a review of the literature.
Galactosemias
Clinical significance of plasma galactose and erythrocyte galactose-1-phosphate measurements in transferase-deficient galactosemia and in individuals with below-normal transferase activity.
Galactosemias
Diagnosis of inherited disorders of galactose metabolism.
Galactosemias
Duarte (DG) galactosemia: a pilot study of biochemical and neurodevelopmental assessment in children detected by newborn screening.
Galactosemias
Galactosemia: when is it a newborn screening emergency?
Galactosemias
GALT Deficiency Galactosemia.
Galactosemias
Hemoglobin A1 in galactosemia, a possible role in monitoring dietary compliance.
Galactosemias
Hereditary Galactosemia.
Galactosemias
Heterozygosity for the classical galactosemia mutation does not affect ovarian reserve and menopausal age.
Galactosemias
Hypoglycosylation with increased fucosylation and branching of serum transferrin N-glycans in untreated galactosemia.
Galactosemias
In vivo metabolism and UTP-depleting action of 2-deoxy-2-fluoro-D-galactose.
Galactosemias
Isoelectrofocusing of erythrocyte galactose 1 phospho uridyl transferase in a family with both galactosemia and Duarte variants.
Galactosemias
Issues on universal screening for galactosemia.
Galactosemias
Misfolding of galactose 1-phosphate uridylyltransferase can result in type I galactosemia.
Galactosemias
Neonatal screening for galactosemia by quantitative analysis of hexose monophosphates using tandem mass spectrometry: a retrospective study.
Galactosemias
Prenatal diagnosis of galactosemia and properties of galactose-1-phosphate uridyltransferase in erythrocytes of galactosemic variants as well as in human fetal and adult organs.
Galactosemias
Prenatal diagnosis of galactosemia.
Galactosemias
Problems in the diagnosis of transferase and galactokinase deficient galactosemia.
Galactosemias
Quantification of galactose-1-phosphate uridyltransferase enzyme activity by liquid chromatography-tandem mass spectrometry.
Galactosemias
Recommendations for newborn screening for galactokinase deficiency: A systematic review and evaluation of Dutch newborn screening data.
Galactosemias
Reversal of aberrant PI3K/Akt signaling by Salubrinal in a GalT-deficient mouse model.
Galactosemias
Simultaneous quantitative estimation of galactose-1-phosphate and galactose in blood for the diagnosis of galactosemia.
Galactosemias
The ability of an LC-MS/MS-based erythrocyte GALT enzyme assay to predict the phenotype in subjects with GALT deficiency.
Galactosemias
The adult galactosemic phenotype.
Galactosemias
The molecular relationship between deficient UDP-galactose uridyl transferase (GALT) and ceramide galactosyltransferase (CGT) enzyme function: a possible cause for poor long-term prognosis in classic galactosemia.
Galactosemias
Unusual presentation of galactosemia in a 4-month-old child.
Galactosemias
[A case of atypical galactosemia(author's transl)]
Galactosemias
[Association of thrombopenia due to platelet alloimmunization with galactosemia in the neonatal period]
Galactosemias
[Effectiveness of the screening programme for galactosemia. New strategy in Poland]
Galactosemias
[Efficacy of dietetic treatment in a case of galactosemia diagnosed late]
Galactosemias
[Late neurologic complications of galactosemia: study of 3 cases]
Genetic Diseases, Inborn
Assessment of galactose-1-phosphate uridyltransferase activity in cells and tissues.
Genetic Diseases, Inborn
Misfolding of galactose 1-phosphate uridylyltransferase can result in type I galactosemia.
Hepatomegaly
Negative screening tests in classical galactosaemia caused by S135L homozygosity.
Neoplasms
Mouse monoclonal antibodies which recognize a human (beta 1-4)galactosyl-transferase associated with tumor in body fluids.
Ovarian Neoplasms
Mutation of galactose-1-phosphate uridyl transferase and its association with ovarian cancer and endometriosis.
Primary Ovarian Insufficiency
Heterozygosity for the classical galactosemia mutation does not affect ovarian reserve and menopausal age.
Pseudotumor Cerebri
Clinical features of galactokinase deficiency: a review of the literature.
Pyuria
Hemolysis due to Alpha-Hemolytic Enterococcus Urinary Infection: A Rare Cause of Early and Severe Unconjugated Hyperbilirubinemia in a Neonate.
Sepsis
Galactosemia: when is it a newborn screening emergency?
Tuberculosis
Identification of amino acids involved in catalytic process of M. tuberculosis GlmU acetyltransferase.
Tuberculosis
Mycobacterium tuberculosis AtsG (Rv0296c), GlmU (Rv1018c) and SahH (Rv3248c) Proteins Function as the Human IL-8-Binding Effectors and Contribute to Pathogen Entry into Human Neutrophils.
udp-glucose-hexose-1-phosphate uridylyltransferase deficiency
Diagnosis and management of galactosemia: an Egyptian experience.
udp-glucose-hexose-1-phosphate uridylyltransferase deficiency
Duarte (DG) galactosemia: a pilot study of biochemical and neurodevelopmental assessment in children detected by newborn screening.
udp-glucose-hexose-1-phosphate uridylyltransferase deficiency
Functional analysis of GALT variants found in classic galactosemia patients using a novel cell-free translation method.
udp-glucose-hexose-1-phosphate uridylyltransferase deficiency
Galactose-1 phosphate uridyl transferase deficiency in the western grey kangaroo (Macropus fuliginosus; marsupialia): a model system for gene therapy studies.
udp-glucose-hexose-1-phosphate uridylyltransferase deficiency
GALT Deficiency Galactosemia.
udp-glucose-hexose-1-phosphate uridylyltransferase deficiency
Hemoglobin A1 in galactosemia, a possible role in monitoring dietary compliance.
udp-glucose-hexose-1-phosphate uridylyltransferase deficiency
Molecular analysis of GALT gene in Argentinian population: Correlation with enzyme activity and characterization of a novel Duarte-like allele.
udp-glucose-hexose-1-phosphate uridylyltransferase deficiency
[Efficacy of dietetic treatment in a case of galactosemia diagnosed late]
udp-glucose-hexose-1-phosphate uridylyltransferase deficiency
[The frequency of uridyl transferase deficiency in patients with galactose intolerance]
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0.011 - 6.66
alpha-D-galactose 1-phosphate
0.092 - 0.5
alpha-D-glucose 1-phosphate
0.051 - 0.324
UDP-alpha-D-galactose
0.0608 - 0.73
UDP-alpha-D-glucose
0.03 - 0.31
UDP-galactose
additional information
additional information
-
0.011
alpha-D-galactose 1-phosphate

-
with UDP-glucose
0.021
alpha-D-galactose 1-phosphate
-
erythrocytes
0.0459
alpha-D-galactose 1-phosphate
mutant F194L, pH 8.8, 37°C
0.084
alpha-D-galactose 1-phosphate
pH 7.5, 50°C
0.09
alpha-D-galactose 1-phosphate
-
galactose grown cells, pH 8.2
0.09
alpha-D-galactose 1-phosphate
-
recombinant mutants P185E, pH 8.7, 37°C
0.1
alpha-D-galactose 1-phosphate
-
glucose grown cells, pH 8.2
0.116
alpha-D-galactose 1-phosphate
-
pH 7.5, 25°C
0.125
alpha-D-galactose 1-phosphate
-
recombinant mutant C160A, pH 8.5, 27°C
0.125
alpha-D-galactose 1-phosphate
recombinant mutant C160A, pH 8.5, 27°C
0.15
alpha-D-galactose 1-phosphate
-
recombinant mutants P185Q, pH 8.7, 37°C
0.2
alpha-D-galactose 1-phosphate
-
pH 8.6, 37°C
0.2
alpha-D-galactose 1-phosphate
-
recombinant mutants P185G, pH 8.7, 37°C
0.223
alpha-D-galactose 1-phosphate
-
recombinant mutant S161A, pH 8.5, 27°C
0.223
alpha-D-galactose 1-phosphate
recombinant mutant S161A, pH 8.5, 27°C
0.288
alpha-D-galactose 1-phosphate
mutant D28Y, pH 8.8, 37°C
0.29
alpha-D-galactose 1-phosphate
-
purified recombinant bifunctional chimeric fusion protein, pH 8.5, 27°C
0.29
alpha-D-galactose 1-phosphate
wild-type, pH 8.8, 37°C
0.3
alpha-D-galactose 1-phosphate
-
recombinant mutant Q168N, pH 8.5, 27°C
0.3
alpha-D-galactose 1-phosphate
-
recombinant wild-type, pH 8.5, 27°C
0.3
alpha-D-galactose 1-phosphate
recombinant wild-type, pH 8.5, 27°C
0.3 - 0.5
alpha-D-galactose 1-phosphate
-
-
0.3 - 0.5
alpha-D-galactose 1-phosphate
-
pH 8.7, 25°C
0.31
alpha-D-galactose 1-phosphate
-
recombinant mutants P185S, pH 8.7, 37°C
0.4
alpha-D-galactose 1-phosphate
-
pH 8.5, 22°C
0.42
alpha-D-galactose 1-phosphate
-
recombinant mutants P185A, pH 8.7, 37°C
0.57
alpha-D-galactose 1-phosphate
-
recombinant wild-type, pH 8.7, 37°C
0.57
alpha-D-galactose 1-phosphate
-
recombinant wild-type, pH 8.7, 42-48°C
0.64
alpha-D-galactose 1-phosphate
-
recombinant mutant S135L, pH 8.7, 42-48°C
0.77
alpha-D-galactose 1-phosphate
recombinant mutant E182A, pH 8.5, 27°C
1.184
alpha-D-galactose 1-phosphate
mutant R333G, pH 8.8, 37°C
1.54
alpha-D-galactose 1-phosphate
-
pH 8.8, 25°C
4
alpha-D-galactose 1-phosphate
-
pH 8.7, 25°C
6.66
alpha-D-galactose 1-phosphate
-
pH 8.7, 25°C
0.092
alpha-D-glucose 1-phosphate

-
pH 7.5, 25°C
0.105
alpha-D-glucose 1-phosphate
-
pH 7.5, 22°C
0.118
alpha-D-glucose 1-phosphate
mutant F194L, pH 8.8, 37°C
0.121
alpha-D-glucose 1-phosphate
mutant D28Y, pH 8.8, 37°C
0.14
alpha-D-glucose 1-phosphate
recombinant mutant C160A, pH 8.5, 27°C
0.14 - 0.16
alpha-D-glucose 1-phosphate
-
-
0.14 - 0.16
alpha-D-glucose 1-phosphate
-
pH 8.7, 25°C
0.159
alpha-D-glucose 1-phosphate
wild-type, pH 8.8, 37°C
0.16
alpha-D-glucose 1-phosphate
recombinant wild-type, pH 8.5, 27°C
0.19
alpha-D-glucose 1-phosphate
recombinant mutant E182A, pH 8.5, 27°C
0.2
alpha-D-glucose 1-phosphate
recombinant mutant S161A, pH 8.5, 27°C
0.202
alpha-D-glucose 1-phosphate
mutant R333G, pH 8.8, 37°C
0.5
alpha-D-glucose 1-phosphate
-
pH 8.8, 25°C
0.051
UDP-alpha-D-galactose

-
pH 7.5, 25°C
0.0897
UDP-alpha-D-galactose
mutant F194L, pH 8.8, 37°C
0.141
UDP-alpha-D-galactose
wild-type, pH 8.8, 37°C
0.179
UDP-alpha-D-galactose
mutant D28Y, pH 8.8, 37°C
0.324
UDP-alpha-D-galactose
mutant R333G, pH 8.8, 37°C
0.0608
UDP-alpha-D-glucose

mutant F194L, pH 8.8, 37°C
0.0816
UDP-alpha-D-glucose
wild-type, pH 8.8, 37°C
0.134
UDP-alpha-D-glucose
-
pH 7.5, 25°C
0.2857
UDP-alpha-D-glucose
mutant R333G, pH 8.8, 37°C
0.4487
UDP-alpha-D-glucose
mutant D28Y, pH 8.8, 37°C
0.73
UDP-alpha-D-glucose
pH 7.5, 50°C
0.03
UDP-galactose

-
pH 7.5, 22°C
0.031
UDP-galactose
-
pH 8.7, 25°C
0.048
UDP-galactose
recombinant mutant E182A, pH 8.5, 27°C
0.062
UDP-galactose
recombinant mutant S161A, pH 8.5, 27°C
0.078
UDP-galactose
recombinant mutant C160A, pH 8.5, 27°C
0.12
UDP-galactose
recombinant wild-type, pH 8.5, 27°C
0.31
UDP-galactose
-
pH 8.8, 25°C
0.015
UDP-glucose

-
erythrocytes
0.02
UDP-glucose
-
pH 8.5, 22°C
0.023
UDP-glucose
-
pH 8.7, 25°C
0.065
UDP-glucose
-
pH 8.6, 37°C
0.072
UDP-glucose
-
recombinant mutant C160A, pH 8.5, 27°C
0.072
UDP-glucose
recombinant mutant C160A, pH 8.5, 27°C
0.08
UDP-glucose
-
purified recombinant bifunctional chimeric fusion protein, pH 8.5, 27°C
0.082
UDP-glucose
-
recombinant mutant C160A, pH 8.5, 27°C
0.082
UDP-glucose
recombinant mutant C160A, pH 8.5, 27°C
0.12
UDP-glucose
-
recombinant mutant S135L, pH 8.7, 42-48°C
0.13
UDP-glucose
-
wild-type from lymphoblasts, 37°C
0.14
UDP-glucose
-
recombinant mutant P185S, pH 8.7, 37°C
0.14
UDP-glucose
-
N314D homozygous mutant from lymphoblasts, 37°C
0.2
UDP-glucose
-
recombinant mutant Q168N, pH 8.5, 27°C
0.2
UDP-glucose
-
recombinant wild-type, pH 8.5, 27°C
0.2
UDP-glucose
recombinant wild-type, pH 8.5, 27°C
0.2
UDP-glucose
-
wild-type from erythrocytes, 37°C
0.2
UDP-glucose
-
recombinant wild-type and mutants P185E and P185A, pH 8.7, 37°C
0.2 - 0.26
UDP-glucose
-
pH 8.7, 25°C
0.2 - 0.26
UDP-glucose
-
pH 8.7, 25°C
0.21
UDP-glucose
-
recombinant wild-type, pH 8.7, 42-48°C
0.34
UDP-glucose
-
recombinant mutants P185G, pH 8.7, 37°C
0.39
UDP-glucose
recombinant mutant E182A, pH 8.5, 27°C
0.4
UDP-glucose
-
recombinant mutants P185Q, pH 8.7, 37°C
0.41
UDP-glucose
-
pH 8.8, 25°C
additional information
additional information

-
-
-
additional information
additional information
-
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics
-
additional information
additional information
-
kinetics at 4°C, wild-type and mutants
-
additional information
additional information
-
kinetics at 4°C, wild-type and mutants
-
additional information
additional information
kinetics at 4°C, wild-type and mutants
-
additional information
additional information
-
mutant H166A
-
additional information
additional information
-
wild-type and mutants in the 2-step reaction
-
additional information
additional information
-
wild-type and mutants in the 2-step reaction
-
additional information
additional information
-
wild-type and mutants in the 2-step reaction
-
additional information
additional information
-
wild-type and mutants in the 2-step reaction
-
additional information
additional information
-
mutant H166G
-
additional information
additional information
-
mutant H166G
-
additional information
additional information
-
kinetics forward and reverse reaction, overview
-
additional information
additional information
-
steady-state kinetics of G, overview
-
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C160S
site-directed mutagenesis, 12fold reduced activity
C52S
site-directed mutagenesis, 3000fold less active than the wild-type, no formation of reaction intermediate UMP-enzyme, low metal content, low expression level
C55S
site-directed mutagenesis, 600fold less active than the wild-type, no formation of reaction intermediate UMP-enzyme, low metal content, low expression level
E182A
site-directed mutagenesis, 50% activity compared to wild-type, normal formation of reaction intermediate UMP-enzyme, contains reduced zinc content and no iron
H115N
site-directed mutagenesis, 2.9% activity compared to wild-type, slightly reduced formation of reaction intermediate UMP-enzyme, retention of zinc and iron
H164N
site-directed mutagenesis, 10000fold less active than the wild-type, no formation of reaction intermediate UMP-enzyme, low metal content, low expression level
H166 A
-
site-directed mutagenesis, point mutation leads to shift of the enzyme activity to UDP-hexose synthase activity, formation of uridine 5'-phosphoimidazolate and alpha-D-glucose 1-phosphate from UDP-glucose and imidazole, highly reduced activity compared to H166G mutant
Q168G
-
site-directed mutagenesis, reduced activity
Q168H
-
site-directed mutagenesis, reduced activity
A101D
mutation identified in a in patient with classic galactosemia
A276N
mutation identified in a in patient with classic galactosemia
D28Y
variant associated with type I galactosemia. 13.5% of wild-type activity
E203K
-
native heterozygous mutant, reduced activity by about 50% in erythrocytes
E291K
-
site-directed mutagenesis for construction of the naturally occuring mutation, 62.8% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
E340X/L218L/N314D
-
native mutant, no or nearly no enzyme activity, L218L is a silent mutation, galactosemia phenotype
F171L
-
site-directed mutagenesis, 10fold decreased activity
F171W
-
site-directed mutagenesis, severely reduced abundance
F171Y
-
site-directed mutagenesis, 4% activity compared to wild-type, no inhibition by excess UDP-glucose
F194L
variant associated with type I galactosemia. 11.9% of wild-type activity
L139P
-
site-directed mutagenesis for construction of the naturally occuring mutation, 1.9% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
L218L/N314D
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native Duarte-1 D1 variant, L218L is a silent mutation, N314D leads to 110-130% activity compared to the wild-type
L74P
variant associated with type I galactosemia, residue is strictly conserved across species. No residual activity
N314D/E203K
-
homozygous N314D mutant with introduced cis mutation E203K does no longer show the reduced, but the full activity and increased thermolablity of mutant without E203K
P183T
-
site-directed mutagenesis for construction of the naturally occuring mutation, 45.2% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
P185A
-
site-directed mutagenesis, reduced activity and reduced expression level compared to wild-type
P185C
-
site-directed mutagenesis, no remaining activity, same expression level compared to wild-type
P185D
-
site-directed mutagenesis, no remaining activity, same expression level compared to wild-type
P185E
-
site-directed mutagenesis, reduced activity, same expression level compared to wild-type
P185F
-
site-directed mutagenesis, no remaining activity, highly reduced expression level compared to wild-type
P185G
-
site-directed mutagenesis, reduced activity and expression level compared to wild-type
P185H
-
site-directed mutagenesis, no remaining activity, reduced expression level compared to wild-type
P185I
-
site-directed mutagenesis, no remaining activity, highly reduced expression level compared to wild-type
P185K
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site-directed mutagenesis, no remaining activity, reduced expression level compared to wild-type
P185L
-
site-directed mutagenesis, no remaining activity, highly reduced expression level compared to wild-type
P185M
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site-directed mutagenesis, no remaining activity, reduced expression level compared to wild-type
P185N
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site-directed mutagenesis, no remaining activity, reduced expression level compared to wild-type
P185Q
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site-directed mutagenesis, reduced activity, increased expression level compared to wild-type
P185R
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site-directed mutagenesis, no remaining activity, same expression level compared to wild-type
P185S
-
site-directed mutagenesis, reduced activity, reduced expression level compared to wild-type
P185T
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site-directed mutagenesis, no remaining activity, reduced expression level compared to wild-type
P185V
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site-directed mutagenesis, no remaining activity, highly reduced expression level compared to wild-type
P185W
-
site-directed mutagenesis, no remaining activity, highly reduced expression level compared to wild-type
P185Y
-
site-directed mutagenesis, no remaining activity, reduced expression level compared to wild-type
P257T
mutation identified in a in patient with classic galactosemia
Q188N
-
site-directed mutagenesis, reduced activity
Q188P
variant identified in a patient with classic galactosemia, introduces a missense substitution near the active site of the GALT enzyme. The variant is found in the compound heterozygous state in a child with classic galactosemia, but not in either of her parents. The patient inherited a common Q188R GALT mutation from the mother
R201H
-
site-directed mutagenesis for construction of the naturally occuring mutation, 62.8% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
R231H
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site-directed mutagenesis for construction of the naturally occuring mutation, below 0.2% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
R258C
-
native mutant, 15-20% activity compared to wild-type, some clinical symptoms
R259W
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site-directed mutagenesis for construction of the naturally occuring mutation, below 0.2% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
R67C
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site-directed mutagenesis for construction of the naturally occuring mutation, 2.3% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
T350A
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site-directed mutagenesis for construction of the naturally occuring mutation, 9.9% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
V151A
-
site-directed mutagenesis for construction of the naturally occuring mutation, 4.6% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucosey
W316X/N314D/G1105C/G1391A
-
native mutant, nearly no enzyme activity, galactosemia phenotype
Y165H
mutation identified in a in patient with classic galactosemia
Y323D
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site-directed mutagenesis for construction of the naturally occuring mutation, 9.6% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
H140F
inactive mutant enzyme
H140G
inactive mutant enzyme
H140N
inactive mutant enzyme
H140F
-
inactive mutant enzyme
-
H140G
-
inactive mutant enzyme
-
H140N
-
inactive mutant enzyme
-
C160A

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-
C160A
site-directed mutagenesis, 18fold reduced activity
H166G

-
H166G
-
site-directed mutagenesis
H166G
active site structure, complex of enzyme with UDP-D-galactose
H166G
-
point mutation leads to shift of the enzyme activity to UDP-hexose synthase activity, formation of uridine 5'-phosphoimidazolate and alpha-D-glucose 1-phosphate from UDP-glucose and imidazole
Q168N

-
-
Q168N
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site-directed mutagenesis, 50fold reduced activity compared to wild-type, 40fold decreased kcat
Q168R

-
site-directed mutagenesis, 270000fold reduced activity compared to wild-type, i.e. nearly no remaining activity, mutant active sites can be uridylated by 65%, with very slow deuridylylation, compared to 100% for the wild-type, reduced metal content
Q168R
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in humans galactosemia causing mutation, used as a model in bacterial system, 30000fold loss of activity, 28% reduced metal ion content
S161A

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-
S161A
site-directed mutagenesis, 7000fold reduced activity compared to wild-type
F171S

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site-directed mutagenesis, no activity
F171S
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site-directed mutagenesis for construction of the naturally occuring mutation, below 0.2% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
F171S
variant associated with type I galactosemia, residue is strictly conserved across species. No residual activity
K285N

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site-directed mutagenesis for construction of the naturally occuring mutation, below 0.2% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
K285N
naturally occuring mutation
K285N
the most common mutations, include p.S135L, p.N314D, p.Q188R, and p.K285N from different ethnicities. Computational pipeline is used to explore the crystal structure and effects due to the most prevalent mutations in the GALT protein. The mutation p.K285N is located in the alpha helix region (alpha4) of the protein is less pathogenic
K285N
the p.K285N allele shows a high frequency in Caucasians and is associated to null blood GALT activity and a severe clinical phenotype
N314D

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site-directed mutagenesis for construction of the naturally occuring mutation, unaltered activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
N314D
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native missense mutant Duarte D, homozygous, characteristic isoform, partial impairment of enzyme activity in human erythrocytes, fibroblasts, and transformed lymphoblasts, reduced Vmax, increased thermal lability
N314D
naturally occuring mutation
N314D
the most common mutations, include p.S135L, p.N314D, p.Q188R, and p.K285N from different ethnicities. Computational pipeline is used to explore the crystal structure and effects due to the most prevalent mutations in the GALT protein. p.N314D mutation, located in the loop region is less pathogenic
N314D/G1105C/G1391A

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native Duarte-2 D2 variant, 40-50% activity compared to the wild-type
N314D/G1105C/G1391A
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native Duarte-2 D2 variant with additional exchange of bases at 1323 G to A, 20-25% activity compared to wild-type, no clinical symptoms
Q188R

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site-directed mutagenesis for construction of the naturally occuring mutation, below 0.2% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
Q188R
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20-30% activity of wild-type activity as heterodimer with wild-type subunit, no activity as homodimer
Q188R
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most common native mutation causing galactosemia in the white population
Q188R
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site-directed mutagenesis for expression of the mutant in Escherichia coli, nearly no activity in vitro
Q188R
-
native mutant, no enzyme activity in vivo, galactosemia phenotype
Q188R
higher tendency of hGALT(p.Q188R) to aggregate due to its reduced ability to be uridylylated
Q188R
significant deviation and fluctuation in the p.Q188R mutation with a loss in compactness reduced the amount of intramolecular hydrogen bonds. p.Q188R mutation (located in the beta-sheet eight region) is extremely pathogenic and has destabilizing effects compared to the native and the other mutations
Q188R
the p.Q188R allele shows a high frequency in Caucasians and is associated to null blood GALT activity and a severe clinical phenotype
R333G

-
native mutant, 20% activity compared to wild-type, no clinical symptoms
R333G
variant associated with type I galactosemia, residue is strictly conserved across species. 0.6% of wild.type activtiy
R333W

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site-directed mutagenesis for construction of the naturally occuring mutation, below 0.2% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
R333W
-
native mutant, catalytically inactive, galactosemic phenotype
R333W
-
20-30% activity of wild-type activity as heterodimer with wild-type subunit, no activity as homodimer
S135L

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site-directed mutagenesis for construction of the naturally occuring mutation, 2.7% of wild-type activity, accumulation of alpha-D-galactose 1-phosphate, UDP-galactose and UDP-glucose
S135L
-
native mutant, can be found in about 50% of galactosemia patients of African-American descent, 10fold reduced enzyme activity compared to wild-type, no steric or electrochemical changes sufficiently close to the active site to result in partial impairment of the reaction
S135L
the mutant demonstrates reduced compactness and increased intramolecular hydrogen bonds. p.S135L mutation (located at the loop region) is extremely pathogenic and has destabilizing effects compared to the native and the other mutations
S135L
the substitution p.S135L is common in Africans and is associated to a mild phenotype albeit having less than 1% residual enzymatic GALT activity
additional information

introduction of Asn and Arg at residue 188, normally Gln, in a three-dimensional model of the Escherichia coli enzyme-UMP-crystal
additional information
-
construction and expression of bifunctional fusion protein composed of galactose-2-phosphate uridylyltransferase and UDP-galactose 4-epimerase with an intervening linker of 3 Ala residues, 20% increased Vmax compared to a mixture of the single enzymes
additional information
-
-
additional information
-
coexpression of wild-type and/or mutant subunits in yeast, study of dimer formation pattern and subunit assortment, mutations: S135L, F171S, F171W, H186G, Q188R, N314D, R333W, overview
additional information
-
Munich2 mutant with base exchange at 2252 G to T, 20-25% activity compared to wild-type, no clinical symptoms
additional information
-
native Schönstadt mutant with base exchange at 897 G to C, 15-20% activity compared to wild-type, some clinical symptoms
additional information
characterization of a large deletion spanning 8489 bp in the GALT gene accounting for the majority of disease alleles in Cypriot patients with classic galactosemia
additional information
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characterization of a large deletion spanning 8489 bp in the GALT gene accounting for the majority of disease alleles in Cypriot patients with classic galactosemia
additional information
construction of transgenic mice expressing a luciferase transgene under control of a 1.9 kb fragment of the UDP-glucose-hexose-1-phosphate uridylyltransferase promotor region , activity is found in most tissues with higher than expected reporter levels in neonatal brain
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