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September 1965

Ciba Foundation Symposium: Control of Glycogen Metabolism.

Arch Neurol. 1965;13(3):332. doi:10.1001/archneur.1965.00470030112014

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Neurological interest in glycogen metabolism centers primarily upon two forms of glycogen storage disease. Pompe's disease affects skeletal muscle and motor neurones in addition to the heart, and McArdle's disease was the first inherited myopathy in which the enzyme defect was recognized. These conditions are discussed at length in this 1963 Ciba Foundation Symposium. Two new neurological conditions are also described: a myopathy due to lack of amylo-1,6-glucosidase (Larner) and a form of hypoglycemia due to lack of glycogen synthetase (Spencer-Peet). Schmid, Manners, Illingworth, Brown, Hers, and Larner provide comprehensive and authoritative discussions of each form of glycogen storage disease. There is an interesting general discussion of such practical considerations as the proper preparation and storage of tissue samples, and the appropriate enzymes to be assayed in new cases. There are several warnings about apparent, but not real, enzyme defects due to artifacts.

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