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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)?

Deficiency of cofactors (B) Signup and view all the answers

What does PDHc deficiency lead to regarding energy production?

High levels of lactic acid (lactic acidosis) (C) Signup and view all the answers

Which symptom is associated with PDHc deficiency in infants?

Respiratory distress (apnea and hypoventilation) (B) Signup and view all the answers

How is PDHc deficiency diagnosed?

Enzyme activity measurement in blood lymphocytes or skin fibroblasts (D) Signup and view all the answers

What management strategy is recommended for PDHc deficiency?

Thiamine supplement and Ketogenic diet (A) Signup and view all the answers

How do PDH Kinase inhibitors affect PDHc?

Activate PDHc by decreasing phosphorylation (A) Signup and view all the answers

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

Provides an alternative fuel source for the brain (A) Signup and view all the answers