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Evidence From Glycogen Storage Diseases

Salvatore DiMauro, MD; Lewis P. Rowland, MD; Paola M. DiMauro, PhD

Arch Neurol. 1970;23(6):534-540.

	Since this article does not have an abstract, we have provided the first 150 words of the full text PDF and any section headings.

LITTLE is known of the mechanisms that control the resting level of glycogen in either normal or pathological human muscle. Animal studies, however, suggest that the polysaccharide itself plays a role in the regulation of its own metabolism by influencing the proportion of active and inactive forms of glycogen synthetase, the principal synthetic enzyme,¹ so that increased tissue levels of glycogen retard synthesis of the polysaccharide. Glycogen inhibits the phosphatase that converts the dependent (D) form of synthetase to the presumably more active independent (I) form.² Similarly, the principal degradative enzyme, phosphorylase, exists in active and inactive forms that are also modified by glycogen. High tissue levels of the polysaccharide increase the proportion of active phosphorylase a at the expense of the inactive phosphorylase b,³ primarily through effects on a separate enzyme, phosphorylase b kinase, that catalyzes the conversion of phosphorylase b to a.⁴ . . . [Full Text PDF of this Article]

Author Affiliations
Philadelphia

From the Neurological Clinical Research Center and the Spiller Neurological Unit, Hospital of the University of Pennsylvania, Philadelphia.

Footnotes
Accepted for publication July 6, 1970.

Reprint requests to Hospital of the University of Pennsylvania, 3400 Spruce St, Philadelphia 19104 (Dr. Rowland).

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