Biochemistry: The Molecular Basis of Life
6th Edition
ISBN: 9780190209896
Author: Trudy McKee, James R. McKee
Publisher: Oxford University Press
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Chapter 9, Problem 7Q
Summary Introduction
To review:
The reason for the occurrence of lactic aciduria.
Introduction:
Pyruvate carboxylase deficiency (PCD) is a disease in which the enzyme pyruvate carboxylase is defective or nonfunctional. The main function of this enzyme is to produce oxaloacetate from pyruvate. An individual with nonfunctional PCD urinates high levels of lactic acid. This is known as lactic aciduria.
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Thiamine is the vitamin precursor for a co-enzyme called thiamine pyrophosphate or TPP.
A patient diagnosed with thiamine deficiency exhibited fatigue and muscle cramps. The muscle cramps have been related to the accumulation of specific metabolic acids. On the following list, circle the metabolic acids that are most likely to accumulate in a thiamine deficiency?
Note: There may be more than one.
Isocitrate
Pyruvate
Succinate
α-ketoglutarate
Malate
Fumarate
(b) Provide a brief explanation for your answer to 1 (a) here
Von Gierke’s disease is also known as glycogen storage disease type I. Patients with von Gierke’s disease lackglucose 6-phosphatase activity. Two prominent symptoms of this disorder are fasting hypoglycemia and lactic acidosis (elevated lactate levels in the blood), especially during strenuous exercise. Explain why these symptoms occur.
What chemical reaction does this enzyme catalyze? Which pathways involve this enzyme? Lacking thisenzyme will cause impairment of which pathways?• Pls consider what pathways are affected by Von Gierke’s disease. Include in your explanation involving the Cori’s cycle.
Von Gierke’s disease is also known as glycogen storage disease type I. Patients with von Gierke’s disease lackglucose 6-phosphatase activity. Two prominent symptoms of this disorder are fasting hypoglycemia and lactic acidosis (elevated lactate levels in the blood), especially during strenuous exercise. Explain why these symptoms occur.
What chemical reaction does this enzyme catalyze? Which pathways involve this enzyme? Lacking thisthe enzyme will cause impairment of which pathways?• Pls consider what pathways are affected by Von Gierke’s disease. Include in your explanation involving Cori’s cycle.
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Chapter 9 Solutions
Biochemistry: The Molecular Basis of Life
Ch. 9 - Prob. 1QCh. 9 - Prob. 2QCh. 9 - Prob. 3QCh. 9 - Prob. 4QCh. 9 - Prob. 5QCh. 9 - Prob. 6QCh. 9 - Prob. 7QCh. 9 - Prob. 1RQCh. 9 - Prob. 2RQCh. 9 - Prob. 3RQ
Ch. 9 - Prob. 4RQCh. 9 - Prob. 5RQCh. 9 - Prob. 6RQCh. 9 - Prob. 7RQCh. 9 - Prob. 8RQCh. 9 - Prob. 9RQCh. 9 - Prob. 10RQCh. 9 - Prob. 11RQCh. 9 - Prob. 12RQCh. 9 - Prob. 13RQCh. 9 - Prob. 14RQCh. 9 - Prob. 15RQCh. 9 - Prob. 16RQCh. 9 - Prob. 17RQCh. 9 - Prob. 18RQCh. 9 - Prob. 19RQCh. 9 - Prob. 20RQCh. 9 - Prob. 21RQCh. 9 - Prob. 22RQCh. 9 - Prob. 23RQCh. 9 - Prob. 24RQCh. 9 - Prob. 25RQCh. 9 - Prob. 26RQCh. 9 - Prob. 27RQCh. 9 - Prob. 28FBCh. 9 - Prob. 29FBCh. 9 - Prob. 30FBCh. 9 - Prob. 31FBCh. 9 - Prob. 32FBCh. 9 - Prob. 33FBCh. 9 - Prob. 34FBCh. 9 - Prob. 35FBCh. 9 - Prob. 36FBCh. 9 - Prob. 37FBCh. 9 - Prob. 38SACh. 9 - Prob. 39SACh. 9 - Prob. 40SACh. 9 - Prob. 41SACh. 9 - Prob. 42SACh. 9 - Prob. 43TQCh. 9 - Prob. 44TQCh. 9 - Prob. 45TQCh. 9 - Prob. 46TQCh. 9 - Prob. 47TQCh. 9 - Prob. 48TQCh. 9 - Prob. 49TQCh. 9 - Prob. 50TQCh. 9 - Prob. 51TQCh. 9 - Prob. 52TQCh. 9 - Prob. 53TQCh. 9 - Prob. 54TQCh. 9 - Prob. 55TQCh. 9 - Prob. 56TQCh. 9 - Prob. 57TQCh. 9 - Prob. 58TQCh. 9 - Prob. 59TQ
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