Miranda A F, Shanske S, Hays A P, DiMauro S
Arch Neurol. 1985 Apr;42(4):371-3. doi: 10.1001/archneur.1985.04060040081017.
Muscle cultures from patients with infantile and later-onset acid maltase deficiency (AMD) and from unaffected controls were studied immunocytochemically with anti-acid maltase (anti-AM) antibodies and fluorescein-labeled goat anti-rabbit IgG second antibody. In control muscle cells, an intense granular distribution of staining was seen, consistent with lysosomal localization of AM. Cultured muscle cells from two patients with infantile AMD (Pompe's disease) did not fluoresce, whereas cells from two patients with AMD of later onset did fluoresce, showing a distribution similar to that of controls.
对患有婴儿型和晚发型酸性麦芽糖酶缺乏症（AMD）的患者以及未受影响的对照者的肌肉培养物，使用抗酸性麦芽糖酶（抗-AM）抗体和荧光素标记的山羊抗兔IgG二抗进行免疫细胞化学研究。在对照肌肉细胞中，可见染色呈强烈的颗粒状分布，这与AM的溶酶体定位一致。两名婴儿型AMD（庞贝病）患者的培养肌肉细胞未发荧光，而两名晚发型AMD患者的细胞则发荧光，显示出与对照相似的分布。
