Structural and mechanistic insight into the basis of mucopolysaccharidosis NIB

Elizabeth Ficko-Blean; Keith A. Stubbs; Oksana Nemirovsky; David J. Vocadlo; Alisdair B. Boraston

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Structural and mechanistic insight into the basis of mucopolysaccharidosis NIB

机译：黏多糖贮积症NIB基础的结构和机制洞察

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Mucopolysaccharidosis III (MPS III) has four forms (A-D) that result from buildup of an improperly degraded glycosaminoglycan in lysosomes. MPS NIB is attributable to the decreased activity of a lysosomal α-N-acetylglucosaminidase (NAGLU). Here, we descri

机译：粘多糖贮积病III（MPS III）具有四种形式（A-D），这是由于溶酶体中降解不当的糖胺聚糖而形成的。 MPS NIB可归因于溶酶体α-N-乙酰氨基葡糖苷酶（NAGLU）活性降低。在这里，我们描述

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来源
《Proceedings of the National Academy of Sciences of the United States of America》 |2008年第18期|6560-6565|共6页
作者
Elizabeth Ficko-Blean; Keith A. Stubbs; Oksana Nemirovsky; David J. Vocadlo; Alisdair B. Boraston;
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收录信息美国《科学引文索引》(SCI);美国《生物学医学文摘》(MEDLINE);美国《化学文摘》(CA);
原文格式 PDF
正文语种 eng
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关键词
Clostridium perfringens; crystal structure; Sanfilippo; hexosaminidase NAGLU;

机译：产气荚膜梭菌;晶体结构Sanfilippo;己糖胺酶NAGLU;

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Structural and mechanistic insight into the basis of mucopolysaccharidosis NIB

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