与II型糖原贮积病（庞贝氏病）相关的来自各种人体组织的酸性α-葡萄糖苷酶的物理化学和免疫学特性

Physico-chemical and immunological properties of acid alpha-glucosidase from various human tissues in relation to glycogenosis type II (Pompe's disease).

作者信息

Koster J F, Slee R G, Van der Klei-Van Moorsel J M, Rietra P J, Lucas C J

出版信息

Clin Chim Acta. 1976 Apr 1;68(1):49-58. doi: 10.1016/0009-8981(76)90287-4.

Abstract

The physico-chemical and immunological properties of acid alpha-glucosidase from various human tissues have been studied. Heat stability of acid alpha-glucosidase from heart, liver and skeletal muscle is identical, but for kidney some different results are obtained. Identical isoelectrofocussing patterns are found for heart, liver and skeletal muscle. Furthermore, the effect of antiserum against human liver acid alpha-glucosidase on the activity of acid alpha-glucosidase from various tissues is studied. The results are discussed in relation to glycogenosis type II (Pompe's disease).

摘要

对来自人体不同组织的酸性α-葡萄糖苷酶的物理化学和免疫学特性进行了研究。心脏、肝脏和骨骼肌中酸性α-葡萄糖苷酶的热稳定性相同，但肾脏的结果有所不同。心脏、肝脏和骨骼肌的等电聚焦图谱相同。此外，研究了抗人肝脏酸性α-葡萄糖苷酶抗血清对不同组织中酸性α-葡萄糖苷酶活性的影响。结合II型糖原贮积病（庞贝氏病）对结果进行了讨论。