There are several congenital disorders of fructose metabolism resulting from a variety of enzyme deficiencies. These patients have difficulty using fructose for a variety of reasons, of course depending on the specific deficient gene product. Fructose is regularly consumed by humans because of their omnivorous diet that includes fruits (…and other sweets). FBPase-1 deficiency is one example of a deficiency that leads to a disorder of fructose metabolism. Explain why these patients present with hypoglycemia and lactic acidosis during fasting. Which one of these will work to treat a FBPase-1 deficient patient and which one will not? Treatments: Lactose Sucrose Ribose Glycerol

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