TREATMENT OF CONGENITAL DISORDERS OF GLYCOSYLATION (CDG) USING MANNOSE

摘要

The invention relates to mannose 1-phosphate derivatives and to pharmaceutical or dietary agents, which contain the same and which are used for treating disorders of glycosylation and, in particular, for treating CDG-1a. These derivatives comprise the following general formula (I), wherein: R<1> and R<2>, which can be identical or different, represent H or formula (II); or R<1> and R<2>, together, represent formula (III) with the provision that only one of the radicals R<1> and R<2> can represent H, and; R<3> and R<4>, which can be identical or different, represent (a), (b), (c) and (d), and radical R<3> can also represent H. Aryl represents an aromatic hydrocarbon radical, which can be substituted by a radical alkyl, and alkyl represents a linear or branched, saturated hydrocarbon radical having 1 to 20 carbon atoms.