Tsuji A, Yang R C, Omura K, Imabayashi T, Suzuki Y
Clin Chim Acta. 1987 Aug 31;167(3):313-20. doi: 10.1016/0009-8981(87)90351-2.
A specific assay for acid alpha-glucosidase in urine was developed to facilitate the diagnosis of glycogenosis II. This enzyme activity was calculated as a difference between the alpha-glucosidase activities before and after immunoprecipitation with antiserum to acid alpha-glucosidase. Acid alpha-glucosidase accounted for 86% of the total activity in control urine. All the cases of various clinical types of glycogenosis II showed either a marked decrease or a complete deficiency of this enzyme activity. A marked decrease of acid alpha-glucosidase was demonstrated by immunoblotting of the urine from patients with late-onset forms of this disease. These results indicate that assays of urinary acid alpha-glucosidase by this immunological method are useful for detection of the various types of glycogenosis II.
开发了一种用于检测尿液中酸性α-葡萄糖苷酶的特定检测方法，以促进糖原贮积症II型的诊断。该酶活性通过用抗酸性α-葡萄糖苷酶抗血清进行免疫沉淀前后的α-葡萄糖苷酶活性差异来计算。酸性α-葡萄糖苷酶占对照尿液总活性的86%。糖原贮积症II型各种临床类型的所有病例均显示该酶活性显著降低或完全缺乏。通过对该疾病迟发型患者尿液的免疫印迹法证实了酸性α-葡萄糖苷酶的显著降低。这些结果表明，通过这种免疫学方法检测尿酸性α-葡萄糖苷酶可用于检测各种类型的糖原贮积症II型。
