Partial characterization and studies of fibroblast and leucocyte neuraminidase activities towards sialyloligosaccharides in adult sialidosis and mucolipidosis II and III

Masaru Kuriyama , Futaba Someya , Tadashi Miyatake , Masai Koseki
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引用次数: 4

Abstract

We described the partial characterization and some properties of fibroblast and leucocyte neuraminidase towards 2 → 3 and 2 → 6 sialyllactose, and 2 → 3 and 2 → 6 sialylhexasaccharide which were isolated from the urine of a patient with adult sialidosis with partial β-galactosidase deficiency. Neuraminidase activities were assayed using the radioactive-labeled derivatives of these saccharide substrates. These neuraminidases (acylneuraminyl hydrolase, EC 3.2.1.18) were partially inactivated by homogenization, sonication and freeze-thawing treatment. The leucocyte neuraminidase was more labile than that of fibroblasts. Fibroblast neuraminidase had about a 10-fold higher activity than leucocyte neuraminidase towards the respective substrates. The neuraminidase from fibroblasts and leucocytes were each able to hydrolyze 2 → 3 isomers 2–3-times faster than 2 → 6 isomers and the sialyllactoses 1.5–3.0-times faster than sialylhexasaccharides. Neuraminidase activities towards all four substrates were deficient in fibroblasts and leucocytes from the patients with adult sialidosis. Loss of activity was especially prominent in fibroblasts, while considerable residual activities (about 20–30%) remained in leucocytes. In mucolipidosis II and III patients, these neuraminidase activities showed normal levels in leucocytes, although they were decreased in fibroblasts. The discrepancy between neuraminidase activities towards 2 → 3 and 2 → 6 isomers was not found in all the cases.

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成纤维细胞和白细胞神经氨酸酶对唾液寡糖在成人唾液病和粘脂病II和III中的活性的部分表征和研究
本文报道了从成人唾液中毒部分β-半乳糖酶缺乏症患者尿液中分离的2→3、2→6唾液基乳糖和2→3、2→6唾液基六糖的成纤维细胞和白细胞神经氨酸酶的部分特征和一些性质。利用这些糖底物的放射性标记衍生物测定神经氨酸酶活性。这些神经氨酸酶(acylneuraminyl hydrolase, EC 3.2.1.18)通过匀浆、超声和冻融处理部分失活。白细胞神经氨酸酶比成纤维细胞更不稳定。成纤维细胞神经氨酸酶对相应底物的活性比白细胞神经氨酸酶高约10倍。来自成纤维细胞和白细胞的神经氨酸酶水解2→3异构体的速度均比2→6异构体快2 ~ 3倍,水解唾液基糖的速度均比唾液基六糖快1.5 ~ 3.0倍。成人唾液中毒患者的成纤维细胞和白细胞对所有四种底物的神经氨酸酶活性都缺乏。活性丧失在成纤维细胞中尤为明显,而白细胞中仍有相当大的活性残留(约20-30%)。在II型和III型粘脂病患者中,这些神经氨酸酶活性在白细胞中显示正常水平,尽管它们在成纤维细胞中下降。神经氨酸酶对2→3和2→6异构体的活性并不存在差异。
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