Updated: 9/18/2020

Fructose Metabolism

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Topic
Overview
  •  FructoseSource
    • 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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