Liver phosphorylase

Deactivation in a child with progressive brain disease, increased hepatic glycogen and increased urinary catecholamines
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      Low activity of hepatic phosphorylase in a girl with degenerative disease of the brain increased to normal after the administration of either glucagon or epinephrine. Hepatic electron micrographs showed increased glycogen in a normal pattern and strikingly different from that in generalized glycogenosis. The surprising finding of a two- to eightfold increase in the excretion of urinary catecholamines led to the study of the effect of continuous epinephrine infusion in dogs. Their activities of liver phosphorylase responded with an initial increase and then with a decline to pre-infusion values or below. Reserpine administered to the child produced a maximal rise in urinary catecholamines which was followed by the lowest phosphorylase measurement recorded.
      The patient's low activity of hepatic phosphorylase did not seem to be the consequence of a deficiency in this enzyme, or of any of the enzymes involved in the phosphorylase system, since proper hormonal activation could be obtained. The low activity might be the expression of a deranged control over the degree of activation of liver phosphorylase. The resulting liver glycogenosis seems unimportant clinically. The implication of a defective control mechanism, however, might have significance in view of the deterioration of the patient's central nervous system. A recent biopsy of the patient's brain disclosed increased glycogen of unusual electron microscopic appearance [14].
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