I-细胞病。关于β-D-N-乙酰己糖胺酶上碳水化合物识别位点结构的一种假说。
I-cell disease. A hypothesis for the structure of the carbohydrate recognition site on beta-D-N-acetylhexosaminidase.
作者信息
Vladutiu G D
出版信息
Biochem J. 1978 May 1;171(2):509-12. doi: 10.1042/bj1710509.
Abstract
I-cell disease (mucolipidosis II) is presented as a model for endo- and exo-cytosis phenomena in man. A hypothesis is presented for the structure of the carbohydrate recognition site on fibroblast-derived beta-D-N-acetylhexosaminidase that may extend to the other affected hydrolases and that is responsible for specific uptake of the enzyme by fibroblasts. The proposed neuraminidase deficiency in I-cell disease is discussed in the light of its significance in influencing the final sugar sequence in the carbohydrate structure of the recognition site.
摘要
I型细胞病（粘脂贮积症II型）被视为人类内吞和外排现象的一个模型。本文提出了一种关于成纤维细胞源性β-D-N-乙酰己糖胺酶上碳水化合物识别位点结构的假说，该假说可能适用于其他受影响的水解酶，并且是成纤维细胞对该酶进行特异性摄取的原因。根据其在影响识别位点碳水化合物结构中最终糖序列方面的重要性，对I型细胞病中拟议的神经氨酸酶缺乏进行了讨论。
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本文引用的文献
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Digestive activity of lysosomes. II. The digestion of macromolecular carbohydrates by extracts of rat liver lysosomes.溶酶体的消化活性。II. 大鼠肝脏溶酶体提取物对大分子碳水化合物的消化作用。
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Physicochemical modifications of lysosomal hydrolases during intracellular transport.溶酶体水解酶在细胞内运输过程中的物理化学修饰。
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3
Analysis of N-acetyl- -D-glucosaminidase in mucolipidosis II (I-cell disease).黏脂贮积症II型（I型细胞病）中N-乙酰-β-D-氨基葡萄糖苷酶的分析。
Clin Chim Acta. 1973 May 18;45(3):243-8. doi: 10.1016/0009-8981(73)90433-6.
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The role of surface carbohydrates in the hepatic recognition and transport of circulating glycoproteins.表面碳水化合物在肝脏对循环糖蛋白的识别和转运中的作用。
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Beta-glucuronidase of bovine liver. Purification, properties, carbohydrate composition.牛肝β-葡萄糖醛酸酶。纯化、性质及碳水化合物组成。
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A recognition marker required for uptake of a lysosomal enzyme by cultured fibroblasts.培养的成纤维细胞摄取溶酶体酶所需的识别标记。
Biochem Biophys Res Commun. 1974 Mar 15;57(1):55-61. doi: 10.1016/s0006-291x(74)80356-6.
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Multiple lysosomal enzyme deficiency due to enzyme leakage?酶泄漏导致多种溶酶体酶缺乏？
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Biochem Biophys Res Commun. 1975 Dec 1;67(3):956-64. doi: 10.1016/0006-291x(75)90768-8.
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