Overview fructose 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 login to view 3 more bullets 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 login to view 7 more bullets treatment login to view 1 more bullet