Glucose-6-phosphatase activity in liver and blood platelets of two patients with glycogen storage disease type I. - Suppr | 超能文献

Glucose-6-phosphatase activity in liver and blood platelets of two patients with glycogen storage disease type I.

作者信息

Oka Y, Mitsuyama T, Nagai B, Arashima S, Ohkubo I, Matsuda I

出版信息

Clin Chim Acta. 1978 Aug 1;87(3):319-26. doi: 10.1016/0009-8981(78)90174-2.

DOI:10.1016/0009-8981(78)90174-2

Abstract

Glucose-6-phosphatase (G-6-Pase) activity in liver and blood platelets of two patients with glycogen storage disease (GSD) type I is described. Both patients had a reduced activity of G-6-Pase in liver. The km value for glucose 6-phosphate (G-6-P) of residual activity in liver of both patients was similar to that of control liver. We could not demonstrate any reduced activity of platelet G-6-Pase in the patients. Platelet G-6-Pase with our assay method seems to represent a nonspecific phosphatase activity. Our observation suggests that it is necessary to examine platelet G-6-Pase of many other patients with GSD type I to confirm that G-6-Pase deficiency can be diagnosed by enzyme assay performed on blood platelets.

摘要

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引用本文的文献

1

Unreliability of platelet glucose-6-phosphatase for the diagnosis of glycogen storage disease type Ia.血小板葡萄糖-6-磷酸酶在糖原贮积病Ia型诊断中的不可靠性。

J Inherit Metab Dis. 1993;16(5):844-50. doi: 10.1007/BF00714276.

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