Questions 1. You decide to try Alex's response to glucagon. This test consists of injecting a high dose of glucagon intravenously and then drawing samples of blood periodically and measuring the glucose content of the samples. After the glucagon injection, Alex's blood sugar rises dramatically. Is this the response you would expect in a normal person? Explain. 2. Liver and muscle biopsies are taken from Alex and analyzed. The biopsies reveal that glycogen content in the liver is normal, but muscle glycogen content is elevated. The biochemical structure of glycogen in both tissues appears to be normal. Suggest some possible explanations for these observations.

Biochemistry
9th Edition
ISBN:9781319114671
Author:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Publisher:Lubert Stryer, Jeremy M. Berg, John L. Tymoczko, Gregory J. Gatto Jr.
Chapter1: Biochemistry: An Evolving Science
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Please answer question 1 and 2. thanks in advanced

Background
Glycogen storage diseases are so named because a hallmark of the diseases is impaired glycogen
storage due to a deficiency of one of the enzymes involved in either glycogen synthesis or glycogen
degradation. Eight kinds of glycogen storage diseases have been identified so far. Each type is
characterized by the lack of a specific enzyme. An understanding of glycogen metabolism is essential in
the proper treatment of this disease, and identification of the deficient enzyme is required before a
treatment protocol can be designed.
Your patient is a fifteen-year-old Caucasian male named Alex K. Alex's mother has brought him to
see you because she is concerned about his inability to perform any kind of strenuous exercise. During
his physical education classes, Alex could not keep up with his classmates and often suffered painful
muscle cramps if he did attempt to exercise. He appeared to be normal if at rest or performing light to
moderate exercise. A physical examination reveals that his liver appears to be normal in size, but his
muscles are flabby and poorly developed. A fasting glucose test showed that Alex was not hypo- or
hyperglycemic. A number of biochemical tests were carried out to identify the type of glycogen storage
disease in this patient.
Questions
1. You decide to try Alex's response to glucagon. This test consists of injecting a high dose of
glucagon intravenously and then drawing samples of blood periodically and measuring the glucose
content of the samples. After the glucagon injection, Alex's blood sugar rises dramatically. Is this
the response you would expect in a normal person? Explain.
2. Liver and muscle biopsies are taken from Alex and analyzed. The biopsies reveal that glycogen
content in the liver is normal, but muscle glycogen content is elevated. The biochemical structure of
glycogen in both tissues appears to be normal. Suggest some possible explanations for these
observations.
Transcribed Image Text:Background Glycogen storage diseases are so named because a hallmark of the diseases is impaired glycogen storage due to a deficiency of one of the enzymes involved in either glycogen synthesis or glycogen degradation. Eight kinds of glycogen storage diseases have been identified so far. Each type is characterized by the lack of a specific enzyme. An understanding of glycogen metabolism is essential in the proper treatment of this disease, and identification of the deficient enzyme is required before a treatment protocol can be designed. Your patient is a fifteen-year-old Caucasian male named Alex K. Alex's mother has brought him to see you because she is concerned about his inability to perform any kind of strenuous exercise. During his physical education classes, Alex could not keep up with his classmates and often suffered painful muscle cramps if he did attempt to exercise. He appeared to be normal if at rest or performing light to moderate exercise. A physical examination reveals that his liver appears to be normal in size, but his muscles are flabby and poorly developed. A fasting glucose test showed that Alex was not hypo- or hyperglycemic. A number of biochemical tests were carried out to identify the type of glycogen storage disease in this patient. Questions 1. You decide to try Alex's response to glucagon. This test consists of injecting a high dose of glucagon intravenously and then drawing samples of blood periodically and measuring the glucose content of the samples. After the glucagon injection, Alex's blood sugar rises dramatically. Is this the response you would expect in a normal person? Explain. 2. Liver and muscle biopsies are taken from Alex and analyzed. The biopsies reveal that glycogen content in the liver is normal, but muscle glycogen content is elevated. The biochemical structure of glycogen in both tissues appears to be normal. Suggest some possible explanations for these observations.
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