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Updated: Dec 25 2021

Fructose Metabolism

  • Overview
    • Source
      • sucrose in fruits and sweeteners
        • disaccharide of glucose and fructose
        • hydrolyzed by sucrase in brush border of small intestine
    • Pathway
      • liver is main site of metabolism
        • also in renal proximal tubule
      • DHAP and glyceraldehyde enter into glycolysis downstream of regulation
    • Clinical relevance
      • fructokinase deficiency
        • benign
        • presentation
          • fructosuria
          • does not have the osmotic pathologies (e.g. cataracts) associated with galactokinase deficiencies
            • fructose is not an aldose and therefore not substrate for aldose reductase
      • fructose 1-P aldolase (aldolase B) deficiency
        • aka hereditary fructose intolerance
        • AR
        • more severe than fructokinase deficiency because fructose 1-P acts as a phosphate sink
        • presentation
          • fructosuria
          • lethargy
          • hypoglycemia
          • liver and proximal renal tubule disorder
            • hyperbilirubinemia
            • hyperuricemia
              • degradation of ADP due to loss of Pi
        • treatment
          • fructose (and sucrose)-free diet
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