Broadhead D M, Butterworth J
Clin Genet. 1978 Jun;13(6):504-10. doi: 10.1111/j.1399-0004.1978.tb01206.x.
The diagnosis of Pompe's disease by the assay of acid alpha-glucosidase in kidney and leucocytes was not previously possible because of the presence of another component which had activity at pH 4.0, but was not deficient in the disease. This problem was resolved either by the use of the inhibitors, turanose, maltose and citrate, or by isoelectric precipitation at pH 5.0, which enabled the estimation of acid alpha glucosidase in kidney and leucocytes.
由于存在另一种在pH 4.0时有活性但在该疾病中不缺乏的成分，以前无法通过检测肾脏和白细胞中的酸性α-葡萄糖苷酶来诊断庞贝病。这个问题通过使用抑制剂松二糖、麦芽糖和柠檬酸盐，或通过在pH 5.0时进行等电沉淀得以解决，这使得能够对肾脏和白细胞中的酸性α-葡萄糖苷酶进行评估。
