Inborn errors of metabolism are disorders resulting from genetic defects of metabolic genes that regulate energy metabolism. Although rare, there 2 reported inborn errors of metabolism affecting the process of gluconeogenesis. The first results in a deficiency in fructose- 1,6 – bisphosphatase (FBPase) and the second results in a deficiency in phosphoenolpyruvate carboxylase (PEPCK). Both deficiencies present with the symptom of hypoglycemia (low blood glucose) early in life during periods of fasting. 

A. What are 3 main sources of glucose normally used to maintain euglycemia (normal blood glucose) in healthy individuals? 

B. Explain how deficiencies in either FBPase or PEPCK can lead to this symptom specifically during periods of fasting? Be sure to reference the sources listed in 1A. Please be brief. 

C. The hypoglycemia associated with PEPCK deficiency is often less frequent and less profound than in FBPase deficiency. Please explain this. (hint-think about the precursors for gluconeogenesis and how they are utilized within the gluconeogenic pathway). Please be brief. ?

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