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Journal of the American Medical Association, 1958
Galactosemia and galactosuria characterize a hereditary disease caused by an inborn error of metabolism, which produces in an affected child the inability to metabolize galactose and lactose normally. Some appropriate terms that have been suggested and used synonymously are galactose diabetes, essential galactosuria, congenital galactosemia, congenital
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Galactosemia and galactosuria characterize a hereditary disease caused by an inborn error of metabolism, which produces in an affected child the inability to metabolize galactose and lactose normally. Some appropriate terms that have been suggested and used synonymously are galactose diabetes, essential galactosuria, congenital galactosemia, congenital
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Endogenous galactose formation in galactose-1-phosphate uridyltransferase deficiency
Archives of Physiology and Biochemistry, 2014Patients with classical galactosaemia (galactose-1-phosphate uridyltransferase (GALT) deficiency) manifest clinical complications despite strict dietary galactose restriction. Therefore the significance of endogenous galactose production has been assessed.
Peter, Schadewaldt +4 more
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Galactose toxicity in the chick: Oxidation of radioactive galactose
Biochimica et Biophysica Acta (BBA) - General Subjects, 1970Abstract Radioactive galactose has been shown to be converted to 14 CO 2 by both the intact chick and by various tissues in vitro . The pattern of 14 CO 2 liberation by the intact chick was not related to the sex of the chick nor to whether the 14 C was in C-1 or C-2 position. [1- 14 C]Glucose was metabolized to 14 CO 2 at a faster rate than [
H J, Wells, M, Gordon, S, Segal
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GALACTOSE STUDIES IN AN INFANT WITH IDIOPATHIC GALACTOSE INTOLERANCE
Pediatrics, 1948Galactose metabolism has been studied in a group of convalescent children and in one patient with galactosuria, hepatomegaly and splenomegaly, and cataracts. The galactose tolerance of the convalescent infants studied following intravenous injection of the sugar was similar to that observed in healthy adults.
L, GREENMAN, J C, RATHBUN
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Galactose disorders: An overview
Journal of Inherited Metabolic Disease, 1990SummaryThere are three separate disorders of galactose metabolism of clinical importance. Galactokinase deficiency mainly causes cataracts which regress without complications providing a galactose‐free diet is started early enough. UDPgalactose‐4‐epimerase deficiency seems extremely rare.
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Galactose-I-Phosphoric Acid in Galactose Metabolism
Nature, 1939The synthesis of α (?)-galactose-l-phosphoric acid has provided a means of examining the constitution of the galactosephosphate accumulating in the liver during galactose assimilation1. Both natural and synthetic esters are non-reducing, very readily hydrolysed by acid, and resistant to alkaline hydrolysis.
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Galactose-sensitive mutants of Salmonella I. Metabolism of galactose
Biochimica et Biophysica Acta, 1961Abstract The metabolism of galactose was studied in peculiar mutants of Salmonella (M mutants) which (1) fail to produce acid from galactose and (2) undergo severe bacteriolysis when grown in the presence of galactose. The mutants were shown to have a specific hereditary defect in the enzyme UDPGal-4-epimerase, and to accumulate intracellularly ...
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Galactose 1-phosphate in galactose cataract
Biochimica et Biophysica Acta, 1955Y, SCHWARZ, L, GOLBERG
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