a) In the human body the glycogen is broken down into glucose by the action of s
ID: 543546 • Letter: A
Question
a) In the human body the glycogen is broken down into glucose by the action of specific enzymes. Due to the deficiency of these enzymes the breakdown of glycogen doesn’t take place and as a result of this glycogen remains in the body. The enzyme responsible in the breakdown is phosphofructokinase and a person suffering from this has a deficiency of PFK. The accumulation of glycogen occurs because it is not broken down to glucose for digestion.
b)Patients with Tarui disease are advised not to engage in strenuous activity to avoid muscle pain and cramping because the energy levels goes down due to absence of glucose and due to production of lactic acid in excess, which leads to cramps and pain.
3. Tarui disease, or Type VIl glycogen storage disease, is characterized by the accumulation of glycogen in skeletal muscles. The disorder is thought to be caused by the loss of muscle phosphofructokinase (PFK) A. Explain how PFK deficiency could lead to glycogen accumulation. (2.5 points) B. Why are patients with Tarui disease advised not to engage in strenuous activity? (2.5 points)Explanation / Answer
3.A.
The defect of enzymes is glycogen storage disease.
In Some cases it is limited to specific tissues.
In most cases it effects on metabolism. It directly effects the glycolysis.
In humans Glycogen enters in the muscles it results breaks the normal glucose it regulates breakdown of glucose.
This causes dramatic rise in ammonia level.
B.Ans.
Strenuous exercise leads to the muscle pain it results the weakness.
Often they follow moderate exercise.
During in excersize ther release protein it is called myoglobinuria.
It results kidney injury.
It leads to increase the production of uric acid then the kidneys are unable to remove the Uric acid. So the moderate excersize is preferred.
Sterenuous excersize leads nausea and vomitings.
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