The storage products in genetic and swainsonine-induced human mannosidosis

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 o...

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Bibliographic Details
Published in:Biochemical journal 1983-12, Vol.215 (3), p.693-696
Main Authors: Cenci di Bello, I, Dorling, P, Winchester, B
Format: Article
Language:English
Subjects:
Alkaloids - pharmacology
alpha-Mannosidase
Carbohydrate Metabolism, Inborn Errors - metabolism
Cells, Cultured
Child
Chromatography, Thin Layer
Fibroblasts - drug effects
Fibroblasts - metabolism
Glycoproteins - metabolism
Humans
Lysosomes - enzymology
Mannose - metabolism
Mannosidases - antagonists & inhibitors
Mannosidases - deficiency
Oligosaccharides - metabolism
Swainsonine
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Summary: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.
ISSN:0264-6021
1470-8728
DOI:10.1042/bj2150693