Results 131 to 140 of about 66,671 (164)

Uridine diphosphate galactose 4-epimerase deficiency

European Journal of Pediatrics, 1981
A case of uridine diphosphate galactose (UDP-Gal) 4-epimerase deficiency was discovered by mass screening of newborn infants. UDP-Gal 4-epimerase activity of red blood cells from the patient was found to be remarkably low, i.e., 7.5% of the level in normal controls at comparable ages.
K, Oyanagi, F, Nakata, S, Hirano, H, Sogawa, N, Takayanagi, R, Minami, S, Tsugawa, T, Nakao, N, Ichihara   +8 more
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Reaction of uridine diphosphate galactose 4-epimerase with a suicide inactivator

Biochemistry, 1990
UDPgalactose 4-epimerase from Escherichia coli is rapidly inactivated by the compounds uridine 5'-diphosphate chloroacetol (UDC) and uridine 5'-diphosphate bromoacetol (UDB). Both UDC and UDB inactivate the enzyme in neutral solution concomitant with the appearance of chromophores absorbing maximally at 325 and 328 nm, respectively. The reaction of UDC
G R, Flentke, P A, Frey
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Erythrocytic uridine diphosphate galactose in galactosaemia

Journal of Inherited Metabolic Disease, 1991
SummaryAn earlier claim of a deficiency of uridine diphosphate galactose in erythrocytes of galactosaemia patients was not confirmed. Enzymic techniques similar to those of the earlier investigators were used to determine not only the concentration of uridine diphosphate galactose but also the ratio of this concentration to the sum of the uridine sugar
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Myelodysplasia and deficiency of uridine diphosphate–galactose 4-epimerase

The Journal of Pediatrics, 1995
A 4-year-old girl known to have peripheral uridine diphosphate-galactose 4-epimerase deficiency was examined for bruising and thrombocytopenia. She had dysplastic peripheral blood and bone marrow changes, with a global platelet function defect. Uridine diphosphate-galactose-4-epimerase participates in a metabolic pathway that provides substrates for ...
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Inhibition of uridine diphosphate glucose dehydrogenase by metabolic intermediates of galactose

Biochimica et Biophysica Acta (BBA) - Specialized Section on Enzymological Subjects, 1964
Abstract UDPG dehydrogenase (EC 1.1.1.22) from calf liver is competitively inhibited by UDPGal in vitro and “uncompetitive” inhibition of the enzyme is shown by UDP. The K i for UDPGal is 1.33 · 10 −4 M, the K m for UDPG is 0.9–1.2 · 10 −5 M at pH 8.3.
G. Salitis, I.T. Oliver
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Roots: Early explorations of the pathways of uridine diphosphate galactose in man and in microorganisms

BioEssays, 1985
AbstractThirty years ago, a number of human inborn errors in carbohydrate metabolism were explored with specific enzymatic tests on blood samples (hemolysates). Hereditary galactosemia was the first example. When the inoperative step in galactose metabolism was specified, the basis for the diet therapy used on the galactosemic infants, namely galactose‐
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Differences in Uridine Diphosphate Galactose-4-Epimerase between Haemolysates of Newborns and of Adults

Nature, 1967
THIS communication presents an observation not yet reported concerning the effect of exogenous nicotinamide adenine dinucleotide (NAD) on the activity of uridine diphosphate galactose-4-epimerase. This enzyme (formerly called galactowaldenase) was first demonstrated in yeast extract by Leloir1.
W G, Ng, G N, Donnell, J E, Hodgman, W R, Bergren   +3 more
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Uridine diphosphate galactose 4-epimerase in human and other mammalian hemolysates

Biochimica et Biophysica Acta (BBA) - Enzymology, 1973
Abstract 1. 1. In contrast to the requirement for exogenous NAD+ in the assay of UDP-galactose 4-epimerase (UDPgalactose epimerase, EC 5.1.3.2), in hemolysates from adults, substantial epimerase activity can be demonstrated in hemolysates from newborn infants without addition of NAD+.
William R. Bergren, Won G. Ng, George N. Donnell   +2 more
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Mutations in Escherichia coli that affect uridine diphosphate glucose pyrophosphorylase activity and galactose fermentation

Biochimica et Biophysica Acta, 1963
Abstract The metabolism of galactose was studied in two galactose-non-fermenting mutants which were isolated from Escherichia coli strain K12 and classified genetically as Group E by Drs. J. and E. M. Lederberg . One of them, W4597, was found to have a single defect in UDPG pyrophosphorylase (UTP: α- d -glucose-1-phosphate uridylyltranserase, EC 2.
T, FUKASAWA, K, JOKURA, K, KURAHASHI
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Efficient One-Pot Synthesis of Uridine Diphosphate Galactose Employing a Trienzyme System

Journal of Agricultural and Food Chemistry
The limited availability of high-cost nucleotide sugars is a significant constraint on the application of their downstream products (glycosides and prebiotics) in the food or pharmaceutical industry. To better solve the problem, this study presented a one-pot approach for the biosynthesis of UDP-Gal using a thermophilic multienzyme system consisting of
Yajing Li, Qi Chen, Siyao Liu, Lin Deng, Shichao Li, Renjun Gao   +5 more
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