一种用于α-1,4-葡萄糖苷酶的微量放射化学测定法及其在II型糖原贮积病（庞贝氏病）评估中的应用。
A micro-radiochemical assay for alpha-1,4-glucosidase and its use in the assessment of type II glycogenosis (Pompe's disease).
作者信息
Nelson P V, Carey W F, Pollard A C
出版信息
Clin Chim Acta. 1977 Jun 15;77(3):337-42. doi: 10.1016/0009-8981(77)90238-8.
An assay for alpha-1,4-glucosidase (acid maltase) activity which is deficient in Pompe's disease is described. The assay can be used to measure the enzyme in cultured skin fibroblasts, cultured amniotic cells and peripheral blood leucocytes. [U-14 C]Maltose is used as the substrate in a total assay volume of 8 microliter. The product, [U-14C]glucose, is separated from the substrate by cellulose thin-layer chromatography. The procedure permits replicate assays from 400 microliter whole blood and from amniotic cells in primary culture. Discrimination of the heterozygous Pompe state appears to be facilitated.
本文描述了一种用于检测α-1,4-葡萄糖苷酶（酸性麦芽糖酶）活性的检测方法，该酶在庞贝病中缺乏。该检测方法可用于测量培养的皮肤成纤维细胞、培养的羊膜细胞和外周血白细胞中的该酶。在8微升的总检测体积中，[U-14C]麦芽糖用作底物。产物[U-14C]葡萄糖通过纤维素薄层层析与底物分离。该方法允许从400微升全血和原代培养的羊膜细胞中进行重复检测。杂合庞贝病状态的鉴别似乎更容易。
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