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Questions and Answers
What is the main cause of Fructosuria?
What is the main cause of Fructosuria?
- Deficiency in Fructose 1-phosphate Aldolase
- Deficiency in Fructokinase (correct)
- High levels of fructose in the blood stream
- Liver damage
What is the result of Fructose Intolerance?
What is the result of Fructose Intolerance?
- High levels of fructose in the blood stream
- Fructose 1-phosphate builds up in the cell (correct)
- Fructose is not phosphorylated
- Renal reabsorption of fructose
What is the primary treatment for Fructose Intolerance?
What is the primary treatment for Fructose Intolerance?
- Medication
- Surgery
- Restricting fructose from the diet (correct)
- Dialysis
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Study Notes
- There are two different inborn errors in metabolism that are connected to fructose metabolism.
- In Fructosuria, Fructokinase is either deficient or is functioning at low levels, and if fructose is not phosphorylated, then it does not get trapped inside and can diffuse out of the cell.
- Fructose concentrations stay high in the blood stream, and levels can get so high that renal reabsorption cannot keep up, and fructose spills into the urine.
- In fructose intolerance (fructosemia), there is a deficiency in Fructose 1-phosphate aldolase, and the fructose is phosphorylated and trapped in the cell, but without Aldolase B, Fructose 1-phosphate cannot be converted to useful glycolytic intermediates.
- Fructose 1-phosphate builds up in the cell, and the energy charge goes down in liver cells.
- People with fructose intolerance usually have liver damage.
- The treatment for this disorder is to restrict fructose from the diet.
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