ALPHA-GLUCOSIDASE DEFICIENCY IN GENERALIZED GLYCOGEN-STORAGE DISEASE (POMPES DISEASE)
ALPHA-GLUCOSIDASE DEFICIENCY IN GENERALIZED GLYCOGEN-STORAGE DISEASE (POMPES DISEASE)
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DOI：
10.1042/bj0860011
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发表时间：
1963-01-01
影响因子：
4.1
通讯作者：
HERS, HG
中科院分区：
文献类型：
--
作者：
HERS, HG
人的肝脏、心脏和骨骼肌含有一种酶，它能将麦芽糖和糖原水解为葡萄糖，并催化麦芽糖转化为糖原。这个[α]-(l[向前箭头]4)-葡萄糖苷酶来自Pompe病（糖原蓄积病的心脏扩张形式）患儿的组织。讨论了麦芽糖酶缺失导致糖原储存的机制。
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.