How much do you know about Aldolase B deficiency?
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Questions and Answers

Which molecule accumulates in Aldolase B deficiency, causing a drop in the level of inorganic phosphate and ATP?

  • Glucose 1-P
  • Glucose 6-P
  • Fructose 6-P
  • Fructose 1-P (correct)
  • What happens to the levels of ATP and AMP in Aldolase B deficiency?

  • ATP falls, AMP rises (correct)
  • Both ATP and AMP fall
  • ATP rises, AMP falls
  • Both ATP and AMP rise
  • What is the consequence of hyperuricemia in Aldolase B deficiency?

  • Gluconeogenesis is prevented
  • Blood clotting factors increase
  • Protein synthesis is enhanced
  • AMP is degraded (correct)
  • What is the effect of the lack of hepatic ATP in Aldolase B deficiency?

    <p>Prevents gluconeogenesis and protein synthesis</p> Signup and view all the answers

    What is the recommended therapy for Aldolase B deficiency?

    <p>Rapid detection and removal of fructose and sucrose from the diet</p> Signup and view all the answers

    What causes hyperuricemia in Aldolase B deficiency?

    <p>Degradation of AMP</p> Signup and view all the answers

    What is the consequence of hypoglycemia with vomiting in Aldolase B deficiency?

    <p>Prevents gluconeogenesis</p> Signup and view all the answers

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