Compounds and methods for treating glycogen storage disease and other pathological conditions resulting from formation of age-proteins

A compound having the structural formula:

• wherein
• X is a divalent moiety selected from the group consisting of —NR′-, —S(O)—, —S(O)2—, or —O—, R′ being selected from the group consisting of H, and linear or branched chain alkyl group (C1–C4) and an unsubstituted or substituted aryl group (C6–C10) and an aralkyl group (C7–C10);
• R is a substituent selected from the group consisting of H, an amino acid residue, said amino acid including said NR′ moiety, a polyaminoacid residue said polyamino acid including said NR′ moiety, a peptide chain, a linear or branched chain aliphatic group (C1–C8), which is unsubstituted or substituted with at least one nitrogen or oxygen-containing substituent, a linear or branched chain aliphatic group (C1–C8), which is unsubstituted or substituted with at least one nitrogen- or oxygen-containing substituent and interrupted by at least one —O—, —NH—, or —NR″- moiety, R″ being linear or branched chain alkyl (C1–C6) and an unsubstituted or substituted aryl group (C6–C10) or aralkyl group (C7–C10), with the proviso that when X represents —NR′-, R and R′, together with the nitrogen atom to which they are attached, may also represent a substituted or unsubstituted heterocyclic ring having from 5 to 7 ring atoms, with at least one of nitrogen and oxygen being the only heteroatoms in said ring, said aryl group (C6–C10) or aralkyl group (C7–C10), and said heterocyclic ring substituents being selected from the group consisting of H, alkyl (C1–C6), halogen, CF3, CN and —O-alkyl (C1–C6);
• R1 is a polyol moiety having 1 to 4 linear carbon atoms;
• Y is a hydroxymethylene moiety —CHOH—;
• Z is selected from the group consisting of —H, O-alkyl (C1–C6), -halogen, —CF3, —CN, —COOH and —SO3H2;
• or its pharmaceutically acceptiable salt or its stereoisomer, except that X—R in the above formula does not represent hydroxyl or thiol.