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* aldolase B
 #133412  
  saonew - 10/29/06 01:50
 
  Which of the following are caused by a genetic deficiency in aldolase B?

A. Fructose intolerance

B. Von Gierke’s disease

C. Hyperammonemia

D. Leber's hereditary optic myopathy

E. McArdle’s disease
 
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* Re:aldolase B
#533628
  pashna23 - 10/29/06 01:50
 
  aa  
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* Re:aldolase B
#533629
  cd45 - 10/29/06 01:51
 
  aa  
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* Re:aldolase B
#533631
  student2000 - 10/29/06 01:51
 
  A..  
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* Re:aldolase B
#533635
  saonew - 10/29/06 01:54
 
  Answer A.
Fructose intolerance is due to a deficiency in fructose 1-phosphate aldolase (aldolase B). This prevents fructose 1-PO4 from being metabolized increasing the intracellular concentration of fructose 1-PO4 and disrupting glucose homeostasis.

B. Von Gierke's disease is due to a deficiency in liver glucose 6-phosphatase, trapping glucose 6-phosphate in the cell. This is the Type I glycogen storage disease.

C. Hyperammonemia is caused to some extent by any urea cycle enzyme deficiency, but carbamoyl phosphate synthetase deficiency is one of the more severe defects.

D. Leber's hereditary optic myopathy is due to a mutation in mitochondrial DNA in the gene for NADH-Q reductase.

E. McArdle's disease is due to a deficiency in muscle glycogen phosphorylase.

 
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