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Inborn errors of metabolism are disorders resulting from genetic defects of metabolic genes that regulate energy...

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Biology

Inborn errors of metabolism are disorders resulting from geneticdefects of metabolic genes that regulate energy metabolism.Although rare, there are 2 reported inborn errors of metabolismaffecting the process of gluconeogenesis. The first results in adeficiency of fructose-1,6-bisphosphate (FBPase) and the secondresults in a deficiency in phosphoenolpyruvate carboxylase (PEPCK).Both deficiencies present with the symptom of hypoglycermia (lowblood glucose) early in life during periods of fasting. a) What arethree main sources of glucose normally used to maintain euglycemia(normal blood glucose) in healthy individuals? b) Explain howdeficiencies in either FBPase or PEPCK can lead to this symptomspecifically during fasting. BE SURE TO REFERENCE THE SOURCES THATYOU LISTED IN PART A. c) The hypoglycemia associated with PEPCK isoften less frequent and less profound than in FBPase deficiency.Please explain this (hint think about the precursors forgluconeogenesis and how they are utilized within thegluconeogenenic pathway).

Answer & Explanation Solved by verified expert
3.6 Ratings (552 Votes)
a the three main sources of glucose are carbohydrates fats and proteins b PEP carboxylase is present in plants the correct name of the enzyme is PEP carboxykinase FBPASe and PEP    See Answer
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