Biosynthesis and maturation of alpha-N-acetylglucosaminidase in normal and Sanfilippo B-fibroblasts.
Biosynthesis and maturation of alpha-N-acetylglucosaminidase in normal and Sanfilippo B-fibroblasts.
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正常和 Sanfilippo B 成纤维细胞中 α-N-乙酰氨基葡萄糖苷酶的生物合成和成熟。
DOI：
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发表时间：
1984
影响因子：
9.8
通讯作者：
J. V. D. van de Kamp
中科院分区：
文献类型：
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作者：
K. von Figura;A. Hasilik;F. Steckel;J. V. D. van de Kamp
通过用[35 S]蛋氨酸标记细胞并通过免疫沉淀分离酶，研究了正常和Sanfilippo B成纤维细胞中α-N-乙酰氨基葡萄糖苷酶的生物合成。通过聚丙烯酰胺凝胶电泳分离免疫沉淀的多肽，并通过荧光法可视化。α-N-乙酰氨基葡糖苷酶作为表观摩尔数的前体合成。重量87000人。前体的细胞内加工产生两种表观摩尔数的多肽。wts. 73，000和76，000通过几个中间体。它在合成后3天内完成。不到30%的新合成的前体被分泌。在10 mM NH 4Cl存在下，分泌增强至80%以上。在我们的研究中，没有α-N-乙酰氨基葡萄糖苷酶多肽可以检测到成纤维细胞的严重或轻度形式的圣菲利波病，B型的影响。
The biosynthesis of alpha-N-acetylglucosaminidase in normal and Sanfilippo B fibroblasts was studied by labeling cells with [35S]methionine and isolation of the enzyme by immunoprecipitation. The immunoprecipitated polypeptides were separated by polyacrylamide gel electrophoresis and visualized by fluorography. alpha-N-acetylglucosaminidase is synthesized as a precursor of an apparent mol. wt. of 87,000. Intracellular processing of the precursor yields two polypeptides of apparent mol. wts. of 73,000 and 76,000 via several intermediates. It is accomplished within 3 days after synthesis. Less than 30% of the newly synthesized precursor is secreted. In the presence of 10 mM NH4Cl, secretion is enhanced to more than 80%. In our study, no alpha-N-acetylglucosaminidase polypeptides could be detected in fibroblasts from patients affected with either the severe or mild form of Sanfilippo disease, type B.