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fructose 1-phosphate fructose derivative hepatic fructokinase mucosa glucose metabolism intermediate dhap dihydroxyacetone phosphate glycolysis gluconeogenesis tca cycle hereditary intolerance hypoglycemia glycogenolysis biosynthetic pathway rendering metabolite biosynthesis mechanism path catabolism biosynthetic biogenesis substrate precursor synthesis bioprecursor metabolic enzyme endogenous contractor human metabolome database hmdb molecule molecular structural structure formula skeletal geometry ball stick