Phosphorylase Kinase of the Liver: Deficiency in a Girl with Increased Hepatic Glycogen

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Science  23 Sep 1966:
Vol. 153, Issue 3743, pp. 1534-1535
DOI: 10.1126/science.153.3743.1534


Studies of a child with glycogenosis revealed an increased concentration of glycogen and low phosphorylase activity in her liver. Using mixtures of homogenates of the patient's liver and of normal liver, we found the low phosphorylase activity to be caused by a deficiency of phosphorylase kinase and not of hepatic phosphorylase. The fact that phosphorylase activity was restored to normal values by the addition of phosphorylase b kinase from rabbit muscle substantiates this conclusion.