Paton B C, Hughes J L, Harzer K, Poulos A
Department of Chemical Pathology, Adelaide Medical Center for Women and Children, Adelaide Children's Hospital Campus, Australia.
Eur J Cell Biol. 1990 Feb;51(1):157-64.
The intracellular localization of sphingolipid activator protein 2 (SAP-2) was determined immunocytochemically using an antiserum raised against a SAP-2 preparation from Gaucher spleen. The immunolabeling indicated that SAP-2 was largely localized in the lysosomes of fibroblasts from normal individuals. In some lysosomes the labeling was greatest around the perimeter of the matrix, suggesting an association between the activator and lysosomal membrane components. The labeling technique was also applied to fibroblasts from a patient with a unique sphingolipid storage disorder. Consistent with immunoblotting studies on electrophoretograms, both the patient and his affected fetal sibling were found to be deficient in immunoreactive SAP-2.
使用针对来自戈谢病脾脏的鞘脂激活蛋白2（SAP - 2）制剂产生的抗血清，通过免疫细胞化学方法确定了鞘脂激活蛋白2（SAP - 2）的细胞内定位。免疫标记表明，SAP - 2主要定位于正常个体成纤维细胞的溶酶体中。在一些溶酶体中，标记在基质周边最为明显，这表明激活剂与溶酶体膜成分之间存在关联。该标记技术也应用于一名患有独特鞘脂贮积症患者的成纤维细胞。与电泳图上的免疫印迹研究一致，发现该患者及其受影响的胎儿同胞均缺乏免疫反应性SAP - 2。
