3. Hers disease results from an absence of liver glycogen phosphorylase and may

Question

3. Hers disease results from an absence of liver glycogen phosphorylase and may results in serious illness. In McArdle disease, muscle glycogen phosphorylase is absent. Although exercise is difficult for patients suffering from McArdle disease, the disease is rare life threatening. Why are there different manifestations of the absence of glycogen phosphorylase in the two tissues? a) What does the existence of these two different diseases indicate about the genetic nature of the phosphorylase? b)

Explanation / Answer

muscle glycogen phosphorylase caused by myophosphorylase. Failure of the enzyme leads to impairs the work of ATPases. Due to lack of normal pH fall during exercise, which impairs the creatine kinase equilibrium and exaggerates the rise of ADP. Also, mutation prevent breakdown of glycogen leads to muscle cells cannot produce enough energy, so muscles become easily fatigued.

Liver glycogen phosphorylase which break down glycogen stored in liver. Deficiency of this enzyme results in
the abnormal accumulation of glycogen in the body. This leads to enlargement of liver, low blood glucose, increase acetone and ketone in blood. liver is the important energy source where glycogen is degraded and give release glucose in the blood.If the enzyme is non functional glucose formation is reduced leads to weakness, hunger, and nervousness and also muscle weakness.

muscle glycogen phosphorylase affect only the muscle if the enzyme not function, but liver glycogen phosphorylase is non functional means it affect whole body.

2. Liver glycogen phosphorylase gene present in autosome. it is autosomal recessive trait. present in chromosome 14.
muscle glycogen phosphorylase gene present in chromosome 11. It also it is autosomal recessive trait.

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