The Subgroups of Type III Glycogenosis
The Subgroups of Type III Glycogenosis
复制标题
III 型糖原增多症的亚组
DOI：
10.1111/j.1432-1033.1967.tb00134.x
复制
发表时间：
1967
期刊：
影响因子：
5.4
通讯作者：
H. Hers
中科院分区：
文献类型：
--
作者：
F. Hoof;H. Hers
在45例III型糖原病患者的肝脏和肌肉活检中测定了糖原的浓度、结构和淀粉-1,6-葡萄糖苷酶的活性。amylo -1,6-葡萄糖苷酶通过磷酸化酶极限糊精形成葡萄糖（全反应）、[14C]葡萄糖并入糖原、低聚葡聚糖（1,4→1,4）转移和单支低聚糖水解来测定。34例被归类为III型A型的基础上完全或几乎完全无活性的酶在两个组织中，无论使用的检测方法。11例病例的特点是amylo-1,6-葡萄糖苷酶的重要残留活性，可以在肝脏或肌肉中检测到，并且只能通过某些方法检测到。
The concentration and the structure of glycogen, and the activity of amylo-1,6-glucosidase have been determined in liver and muscle biopsies of 45 patients affected by type III glycogenosis. Amylo-1,6-glucosidase was measured by the formation of glucose from a phosphorylase limit dextrin (overall reaction), by the incorporation of [14C]glucose into glycogen, by the (1,4 → 1,4) transfer of an oligoglucan and by the hydrolysis of singly branched oligosaccharides. 34 cases were classified as type III A on the basis of a complete or nearly complete inactivity of the enzyme in the two tissues whatever the method used for its detection. 11 cases were characterized by an important residual activity of amylo-1,6-glucosidase which could be detected in the liver or in the muscle and by only some of the methods used.