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 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.
D000470:Alkaloids / Q000494:pharmacology*; D002239:Carbohydrate Metabolism, Inborn Errors / Q000378:metabolism*; D002478:Cells, Cultured; D002648:Child; D002855:Chromatography, Thin Layer; D005347:Fibroblasts / Q000187:drug effects / Q000378:metabolism; D006023:Glycoproteins / Q000378:metabolism; D006801:Humans; D008247:Lysosomes / Q000201:enzymology; D008358:Mannose / Q000378:metabolism*; D008361:Mannosidases / Q000037:antagonists & inhibitors / Q000172:deficiency*; D009844:Oligosaccharides / Q000378:metabolism*; D017026:Swainsonine; D043323:alpha-Mannosidase
D000470:Alkaloids; D006023:Glycoproteins; D009844:Oligosaccharides; D008361:Mannosidases; D043323:alpha-Mannosidase; D008358:Mannose; D017026:Swainsonine