Zeigler M, Bach G
Biochem J. 1981 Sep 15;198(3):505-8. doi: 10.1042/bj1980505.
The cellular localization of glycoprotein and ganglioside sialidases in normal and I-cell-disease cultured fibroblasts has been investigated. Cellular organelles have been separated on a colloidal silica gradient. The subcellular distribution of these enzymes indicated that the glycoprotein sialidase is mainly a lysosomal hydrolase, whereas the ganglioside sialidase is primarily located in the plasma membranes. The latter isoenzymes is tightly bound to these membranes and thus could not be extracted by homogenization in the presence of Triton X-100. The interpretation of this finding and its relation to the pathochemistry of sialidase-deficient disorders is discussed.
已对正常和I细胞病培养成纤维细胞中糖蛋白和神经节苷脂唾液酸酶的细胞定位进行了研究。利用胶体二氧化硅梯度分离细胞器。这些酶的亚细胞分布表明，糖蛋白唾液酸酶主要是一种溶酶体水解酶，而神经节苷脂唾液酸酶主要位于质膜中。后一种同工酶与这些膜紧密结合，因此在Triton X-100存在下通过匀浆无法提取。讨论了这一发现的解释及其与唾液酸酶缺乏症病理化学的关系。
