Regular ArticleGalactose Metabolism in Mice with Galactose-1-Phosphate Uridyltransferase Deficiency: Sucklings and 7-Week-Old Animals Fed a High-Galactose Diet
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Galactose in human metabolism, glycosylation and congenital metabolic diseases: Time for a closer look
2021, Biochimica et Biophysica Acta - General SubjectsCitation Excerpt :The galactonate pathway was first discovered in mammals in 1966 from in vitro studies conducted on healthy rat liver [38]. Later, accumulation of D-galactonate (or D-galactonic acid) was detected also in other tissues, including rat intestine, heart and kidney when rats were maintained on a prolonged high-galactose diet [39–41]. In Guinea pigs high-galactose diet led to accumulation of D-galactono-1,4-lactone in lens, testis, circulation and muscles when galactitol production was suppressed [42,43].
Partial replacement of glucose by galactose in the post-weaning diet improves parameters of hepatic health
2019, Journal of Nutritional BiochemistryAlterations of galactose metabolism caused by deficit of galactose-1-phosphate uridylyltransferase activity: An overview of galactosemia type I
2019, Molecular Nutrition CarbohydratesThe galactose-induced decrease in phosphate levels leads to toxicity in yeast models of galactosemia
2017, Biochimica et Biophysica Acta - Molecular Basis of DiseaseCitation Excerpt :One of the hallmarks of classic galactosemia is the intracellular accumulation of galactose-1-phosphate. Although there are evidences that not all negative outcomes correlates with galactose-1-phosphate accumulation in patients [6–9] and in other models of classic galactosemia [10,11], it is believed that the accumulation of this metabolite is relevant to the pathophysiology of this disease in a still elusive manner [12]. Galactose-1-phosphate is proposed to act as an inhibitor of a series of enzymatic activities such as phosphoglucomutase, glucose-6-phosphate phosphatase, glucose-6-phosphate dehydrogenase, glycogen phosphorylase, UDP-glucose pyrophosphorylase and inositol monophosphatase [12–14].
Pediatric Liver Disease and Inherited, Metabolic, and Developmental Disorders of the Pediatric and Adult Liver
2009, Surgical Pathology of the GI Tract, Liver, Biliary Tract and Pancreas
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