Okada S, Handa M, Hashimoto T, Nishimoto J, Inui K, Furukawa M, Furuyama J, Yabuuchi H, Tate M, Gasa S
Department of Pediatrics, Osaka University Hospital, Japan.
Biochem Int. 1988 Aug;17(2):375-83.
Lymphoblastoid cells transformed by Epstein-Barr virus from peripheral lymphocytes of normal individuals and I-cell disease (ICD) patients were used for the enzymic study of lysosomal hydrolases and N-acetylglucosamine 1-phosphotransferase. ICD lymphoblastoid cells secreted a larger amount of hydrolases into medium than normal cells, although the intracellular hydrolases were not deficient in ICD cells. The stimulating effect of 10 mM ammonium chloride on secretion of hydrolases was found only with normal cells, and not with ICD cells, indicating that the hydrolase molecule bearing mannose 6-phosphate was secreted. The ICD lymphoblastoid cells retained the enzymologic characteristics of both lysosomal hydrolases and N-acetylglucosamine 1-phosphotransferase seen in ICD fibroblasts, which allows us to study the pathophysiology of ICD in cells other than fibroblasts.
利用从正常个体外周淋巴细胞和I细胞病（ICD）患者外周淋巴细胞中经爱泼斯坦-巴尔病毒转化的淋巴母细胞，对溶酶体水解酶和N-乙酰葡糖胺1-磷酸转移酶进行酶学研究。ICD淋巴母细胞向培养基中分泌的水解酶量比正常细胞多，尽管ICD细胞内的水解酶并不缺乏。仅在正常细胞中发现10 mM氯化铵对水解酶分泌有刺激作用，而在ICD细胞中未发现，这表明携带6-磷酸甘露糖的水解酶分子被分泌了。ICD淋巴母细胞保留了ICD成纤维细胞中所见的溶酶体水解酶和N-乙酰葡糖胺1-磷酸转移酶的酶学特征，这使我们能够在成纤维细胞以外的细胞中研究ICD的病理生理学。
