Okada S, Inui K, Furukawa M, Midorikawa M, Nishimoto J, Yabuuchi H, Kato T, Watanabe M, Gasa S, Makita A
Department of Pediatrics, Osaka University Hospital, Japan.
Enzyme. 1987;38(1-4):267-72. doi: 10.1159/000469215.
Since we observed the normalization of intracellular hydrolases in some cell lines of I-cell disease (ICD) by 88 mmol/l sucrose, we have hypothesized that the degree of responses of the hydrolases might be due to biochemical heterogeneity among ICD. In this study the changes of intracellular lysosomal enzymes as well as Golgi enzymes including N-acetylglucosaminyl phosphotransferase (GlcNAcPTase) and extracellular hexosaminidase (HEX) were investigated using normal and ICD fibroblasts. Sucrose loading induced the activities of intracellular HEX and GlcNAcPTase simultaneously only in responding-type ICD cells, and not in nonresponding-type ICD cells, indicating that two biochemical heterogeneous groups exist in ICD.
由于我们观察到在88 mmol/l蔗糖作用下，I细胞病（ICD）某些细胞系中的细胞内水解酶实现了正常化，我们推测水解酶的反应程度可能归因于ICD之间的生化异质性。在本研究中，我们使用正常和成I细胞病的成纤维细胞，研究了细胞内溶酶体酶以及包括N-乙酰葡糖胺磷酸转移酶（GlcNAcPTase）在内的高尔基体酶和细胞外己糖胺酶（HEX）的变化。蔗糖加载仅在反应型ICD细胞中同时诱导了细胞内HEX和GlcNAcPTase的活性，而在无反应型ICD细胞中则未诱导，这表明ICD中存在两个生化异质组。
