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Science 23 September 1966:
Vol. 153. no. 3743, pp. 1534 - 1535
DOI: 10.1126/science.153.3743.1534
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Articles

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

George Hug 1, William K. Schubert 1, and Gail Chuck 1

1 Children's Hospital Clinical Research Center and Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, Ohio

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.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
A New Variant of Glycogen Storage Disease: Type IXc.
A. Lerner, T. C. Iancu, N. Bashan, R. Potashnik, and S. Moses (1982)
Arch Pediatr Adolesc Med 136, 406-410
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