This article reviews synthesis and structures of carbaglycosylamines, a group of carbocyclic sugar analogues. Some unsaturated derivatives are known to be potent glycosidase inhibitors. Among them, N-octyl-4-epi-??-valienamine as a lysosomal ??-galactosidase inhibitor is currently undergoing a new molecular therapeutic trial (chemical chaperone therapy) for control of the human ??-galactosidase deficiency disorder, GM1-gangliosidosis.
Keywords: Carbasugars, aminocyclitols, 5a-carbaglycosylamines, glycosidase inhibitors, chemical chaperone therapy, lysosomal disease, GM1-gangliosidosis
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