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遗传性和苦马豆素诱导的人类甘露糖苷贮积症中的储存产物。

The storage products in genetic and swainsonine-induced human mannosidosis.

作者信息

Cenci di Bello I, Dorling P, Winchester B

出版信息

Biochem J. 1983 Dec 1;215(3):693-6. doi: 10.1042/bj2150693.

DOI:10.1042/bj2150693
PMID:6419731
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1152453/
Abstract

Swainsonine induces the accumulation of mannose-rich oligosaccharides in human fibroblasts. The composition of the storage products shows that swainsonine completely inhibits lysosomal alpha-D-mannosidase and alters processing of glycoproteins by inhibiting Golgi alpha-D-mannosidase II. Comparison of the storage products in genetic and swainsonine-induced mannosidosis suggests that human fibroblasts contain a lysosomal alpha-D-mannosidase that is unaffected in genetic mannosidosis.

摘要

苦马豆素可诱导人成纤维细胞中富含甘露糖的寡糖积累。储存产物的组成表明，苦马豆素可完全抑制溶酶体α-D-甘露糖苷酶，并通过抑制高尔基体α-D-甘露糖苷酶II改变糖蛋白的加工过程。对遗传性和苦马豆素诱导的甘露糖苷贮积症中储存产物的比较表明，人成纤维细胞含有一种在遗传性甘露糖苷贮积症中未受影响的溶酶体α-D-甘露糖苷酶。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0a21/1152453/1194c8741778/biochemj00340-0253-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0a21/1152453/08b33b2ae10b/biochemj00340-0252-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0a21/1152453/1194c8741778/biochemj00340-0253-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0a21/1152453/08b33b2ae10b/biochemj00340-0252-a.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0a21/1152453/1194c8741778/biochemj00340-0253-a.jpg

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Canine alpha-L-fucosidase in relation to the enzymic defect and storage products in canine fucosidosis.犬α-L-岩藻糖苷酶与犬岩藻糖苷贮积症中的酶缺陷及贮存产物的关系
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The catabolism of mammalian glycoproteins. Comparison of the storage products in bovine, feline and human mannosidosis.哺乳动物糖蛋白的分解代谢。牛、猫和人类甘露糖苷贮积症中储存产物的比较。
Biochem J. 1983 Dec 1;215(3):573-9. doi: 10.1042/bj2150573.
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The uptake of swainsonine, a specific inhibitor of alpha-D-mannosidase, into normal human fibroblasts in culture.一种α-D-甘露糖苷酶的特异性抑制剂——苦马豆素在培养的正常人成纤维细胞中的摄取。
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