α-Glucosidase deficiency in generalized glycogen-storage disease (Pompe's disease)
- 1 January 1963
- journal article
- research article
- Published by Portland Press Ltd. in Biochemical Journal
- Vol. 86 (1), 11-16
- https://doi.org/10.1042/bj0860011
Abstract
Human liver and heart and skeletal muscles contain an enzyme that hydrolyses maltose and glycogen into glucose and which catalyses transglucosylation from maltose to glycogen. This [alpha]-(l[forward arrow] 4)-gluco-sidase is about from the tissues of children affected by Pompe''s disease (cardiomegalic form of glycogen-storage disease). The mechanism by which the absence of maltase can cause glycogen storage is discussed.Keywords
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