与II型糖原贮积病（庞贝氏病）相关的来自各种人体组织的α-葡萄糖苷酶的特性

Properties of the alpha-glucosidase from various human tissues in relation to glycogenosis type II (Pompe's disease).

作者信息

Soyama K, Ono E, Shimada N, Tanaka K, Oya N

出版信息

Clin Chim Acta. 1977 Aug 1;78(3):473-8. doi: 10.1016/0009-8981(77)90080-8.

Abstract

The physico-chemical and electrophoretical properties of alpha-glucosidases from various human tissues and urine have been studied. There were some differences among Peak I enzymes (neutral alpha-glucosidases) obtained from liver, heart, muscle, kidney and urine. These differences are based on different effects of tris(hydroxymethyl)aminomethane and various thermostabilities of the Peak I enzymes. Electrophoretically, the Peak I enzyme activity at pH 6.5 from control tissues displayed a two-banded pattern except in kidney and urine. In the patient with the adult form of Pompe's disease the faster band of the Peak I enzyme from heart and muscle was not found and the slower band of the Peak I enzyme from liver was more cathodic. The results are discussed in relation to glycogenosis type II (Pompe's disease).

摘要

对来自人体各种组织和尿液中的α-葡萄糖苷酶的物理化学和电泳性质进行了研究。从肝脏、心脏、肌肉、肾脏和尿液中获得的I峰酶（中性α-葡萄糖苷酶）之间存在一些差异。这些差异基于三（羟甲基）氨基甲烷的不同作用以及I峰酶的各种热稳定性。在电泳中，除了肾脏和尿液外，来自对照组织的pH 6.5时的I峰酶活性呈现出两条带的模式。在患有成人型庞贝病的患者中，未发现来自心脏和肌肉的I峰酶的较快条带，而来自肝脏的I峰酶的较慢条带更偏向阴极。结合II型糖原贮积病（庞贝病）对结果进行了讨论。