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Lab3 - Inborn Errors of Mitochondria Disorders Quiz
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Lab3 - Inborn Errors of Mitochondria Disorders Quiz

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Questions and Answers

What are the products generated when NAD+ undergoes a reaction?

  • CO2, Acetyl CoA, NADH+H+ (correct)
  • Lactic acid, Alanine, ATP
  • ATP, Pyruvate, NAD+
  • Ketone bodies, Thiamine, Acetyl CoA

    • Which of the following inhibits the activity of PDHc?

  • High Acetyl CoA and NADH (correct)
  • ADP and ATP
  • Thiamine deficiency
  • Pyruvate and NAD+

    • What is the consequence of PDHc being phosphorylated?

  • It becomes inactive (correct)
  • It stimulates pyruvate production
  • It becomes more efficient
  • It generates more ATP

    • Which deficiency leads to a lack of Thiamine (vitamin B1)?

    <p>Deficiency of cofactors</p> Signup and view all the answers

    What does PDHc deficiency lead to regarding energy production?

    <p>High levels of lactic acid (lactic acidosis)</p> Signup and view all the answers

    Which symptom is associated with PDHc deficiency in infants?

    <p>Respiratory distress (apnea and hypoventilation)</p> Signup and view all the answers

    How is PDHc deficiency diagnosed?

    <p>Enzyme activity measurement in blood lymphocytes or skin fibroblasts</p> Signup and view all the answers

    What management strategy is recommended for PDHc deficiency?

    <p>Thiamine supplement and Ketogenic diet</p> Signup and view all the answers

    How do PDH Kinase inhibitors affect PDHc?

    <p>Activate PDHc by decreasing phosphorylation</p> Signup and view all the answers

    What is the significance of a Ketogenic diet in managing PDHc deficiency?

    <p>Provides an alternative fuel source for the brain</p> Signup and view all the answers

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