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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

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

Human Anatomy & Physiology (11th Edition)
Human Anatomy & Physiology (11th Edition)
11th Edition
ISBN:9780134580999
Author:Elaine N. Marieb, Katja N. Hoehn
Publisher:Elaine N. Marieb, Katja N. Hoehn
Chapter1: The Human Body: An Orientation
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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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