Page 140 - BIOCHEMISTRY II
P. 140
• Disorders of fructose metabolism: genetic deficiency
of F-1-P aldolase leads to hereditary fructose
intolerance (HFI); this appears when a baby is weaned
and begins to be fed sucrose or fructose-containing
food.
• In this condition, F-1-P accumulates trapping phosphate
in an unusable form that does not return to the general
phosphate pool; this results in a drop in hepatic ATP
level slowing down gluconeogenesis. In addition, the lack
of available phosphate inhibits glycogenolysis. The
inhibition of gluconeogenesis & glycogenolysis leads to
hypoglycemia.
• HFI is managed by the removal of sucrose & fructose
from the diet to prevent liver failure and possible
death.
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