Cell surface-associated lysosomal enzymes in cultured human skin fibroblasts.
Cell surface-associated lysosomal enzymes in cultured human skin fibroblasts.
复制标题
培养的人皮肤成纤维细胞中细胞表面相关的溶酶体酶。
DOI：
10.1016/0014-4827(79)90004-1
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发表时间：
1979
影响因子：
3.7
通讯作者：
B. Voss
中科院分区：
文献类型：
--
作者：
K. von Figura;B. Voss
从完整人皮肤成纤维细胞β-N-乙酰氨基葡萄糖苷酶表面释放的胰蛋白酶。在4个对照细胞系和14个粘多糖沉积病细胞系中，胰蛋白酶可去除的β-N-乙酰氨基葡萄糖苷酶的量相当于细胞内活性的1.5%（范围0.5-4.3%）。在形成溶酶体酶的粘脂沉积症II和III型成纤维细胞中，细胞表面相关β-N-乙酰氨基葡萄糖苷酶不存在，而在β-N-乙酰氨基葡萄糖苷酶缺陷型成纤维细胞（Sandhoff病）中，溶酶体酶与成纤维细胞的细胞表面受体结合缺陷。用单特异性抗血清进行的间接免疫荧光显示，在成纤维细胞的细胞表面上存在β-N-乙酰氨基葡萄糖苷酶、α-N-乙酰氨基葡萄糖苷酶、α-甘露糖苷酶和β-葡萄糖醛酸酶，而在粘脂沉积症II型和III型成纤维细胞的细胞表面上不存在这些酶。β-葡萄糖醛酸苷酶和β-N-乙酰氨基葡萄糖苷酶的同时染色显示，在同一细胞的几乎相同区域存在两种酶。在β-N-乙酰氨基葡萄糖苷酶、α-N-乙酰氨基葡萄糖苷酶（粘多糖沉积病III B）、α-甘露糖苷酶（甘露糖苷沉积病）和β-葡萄糖醛酸酶（粘多糖沉积病VII）缺乏的成纤维细胞的细胞表面存在交叉反应物质。溶酶体酶在细胞表面上的证明与以下假设一致：在成纤维细胞中，溶酶体酶向溶酶体器的转运涉及溶酶体酶经由细胞表面的循环。
Trypsin released from the surface of intact human skin fibroblasts β-N-acetylglucosaminidase. The amount of trypsin removable β-N-acetylglucosaminidase in 4 control and 14 mucopolysaccharidosis cell lines was equivalent to 1.5% (range 0.5–4.3%) of the intracellular activity. Cell surface-associated β-N-acetylglucosaminidase was absent in mucolipidosis II and III fibroblasts that form lysosomal enzymes defective in binding to the cell surface receptors of fibroblasts and in β-N-acetylglucosaminidase deficient fibroblasts (Sandhoff's disease). Indirect immunofiuorescence with monospecific antisera allowed the demonstration of β-N-acetylglucosaminidase, α-N-acetylglucosaminidase, α-mannosidase and β-glucuronidase on the cell surface of fibroblasts, whereas these enzymes were absent on the cell surface of mucolipidosis II and III fibroblasts. Simultaneous staining for β-glucuronidase and β-N-acetylglucosaminidase showed presence of both enzymes in almost identical areas of the same cell. Cross-reacting material was present on the cell surface of fibroblasts with a deficiency of β-N-acetylglycosaminidase, α-N-acetylglucosaminidase (mucopolysaccharidosis III B), α-mannosidase (mannosidosis) and β-glucuronidase (mucopolysaccharidosis VII). The demonstration of lysosomal enzymes on the cell surface is in agreement with the hypothesis that in fibroblasts transport of lysosomal enzymes to the lysosomal apparatus involves cycling of lysosomal enzymes via the cell surface.