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[III型糖原贮积病(界限糊精贮积病)肝脏糖原代谢的研究]

[Study of glycogen metabolism in the liver in type III glycogenosis (limit dextrinosis)].

作者信息

Chibisov I V, Leont'ev A F, Rozenfel'd E L

出版信息

Vopr Med Khim. 1975 Mar-Apr;21(2):162-5.

PMID:166513
Abstract

Data on biochemical study of a patient with glycogenosis of the III type (limit dextrinosis) are presented. In a punctate of liver tissue absence of amylo-1,6-glucosidase activity and significant accumulation of glycogen, which was anomalous in structure, were noted. Loading with galactose and adrenaline caused alterations typical for the III type of glycogenosis. Content of glucose and lactate in blood were also studied in response to the peroral administration of glucose and protein. In erythrocytes of the patient the polysaccharide structure was shown to be anomalous; it resembled the structure of a polysaccharide from liver tissue of the patient.

摘要

本文呈现了一名III型糖原贮积病(界限糊精贮积病)患者的生化研究数据。在肝脏组织穿刺检查中,发现缺乏淀粉-1,6-葡萄糖苷酶活性,且糖原大量蓄积,其结构异常。给予半乳糖和肾上腺素负荷后出现了III型糖原贮积病的典型变化。还研究了口服葡萄糖和蛋白质后血液中葡萄糖和乳酸的含量。该患者红细胞中的多糖结构显示异常;它类似于该患者肝脏组织中多糖的结构。

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Some cases of Type III glycogen storage disease.III型糖原贮积病的一些病例。
Clin Chim Acta. 1976 Mar 1;67(2):123-30. doi: 10.1016/0009-8981(76)90250-3.

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