Biochemistry: The Molecular Basis of Life
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 6Q
Summary Introduction

To review:

Determining the structure of 2-hydroxyglutarate by reviewing the structures of isocitrate and alpha-ketoglutarate.

Introduction:

The citric acid cycle (or Krebs cycle) is a biochemical pathwa, yhich involves a series of enzyme-catalyzed reactions that results in the generation of high-energy compounds. Isocitrate and alpha-ketoglutarate are two of its intermediate products. The normal enzyme, isocitrate dehydrogenase (IDH), converts isocitrate into alpha-ketoglutarate. However, the mutated enzyme IDH1 converts it to 2- hydroxyglutarate. This enzyme is present in high concentration in patients suffering from brain cancer (glioblastoma).

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A mutated IDH1 isoenzyme is found in a high percentage of a type of brain cancer called glioblastoma. Instead of converting isocitrate to α-ketoglutarate, mutated IDH1 converts its substrate to 2-hydroxyglutarate, a circumstance that disrupts the citric acid cycle, among other effects. Review the structures of isocitrate and α-ketoglutarate and determine the structure of 2- hydroxyglutarate
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. can you please do not write by your hand? I mean computer if you can. thank you
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