APPLICATION OF A NITRO-OXY DERIVATIVE MEDICINE FOR THE TREATMENT OF MUSCULAR DYSTROPHY

摘要

1. The use of nitric oxide releasing compounds of formula (Ia)! ! or their enantiomers or diastereomers for the preparation of drugs for the treatment of muscular dystrophies, where in the general formula (Ia) M, X and Y have the following meanings:! M - residue selected from:! ,! where RA is a hydrogen atom or -C (O) CH3,! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! ! X is —O—, —S— or —NR1—, where R1 is H or a linear or branched C1-C6 alkyl; ! Y is a divalent radical having the following meanings:! a) straight or branched C1-C20 alkylene, preferably C1-C10, optionally substituted with one or more substituents selected from the group consisting of: halogen atom, hydroxy, -ONO2 or T, where T is -OC (O) (C1- C10 alkyl) -ONO2 or -O (C1-C10 alkyl) -ONO2; more preferably Y is —C1 -C10 alkylene; ! -C5-C7 cycloalkylene optionally substituted with a linear or branched C1-C10 alkyl group, preferably CH3; ! b)! c)! where n is an integer from 0 to 20, preferably n is an integer from 0 to 5, more preferably n is 0; ! n1 is an integer from 1 to 20, preferably n1 is an integer from 1 to 5, more preferably n1 is 1; ! with the proviso that when Y is selected from divalent radicals indicated under b) and c), then the —ONO2 group in the formula (I) is bonded to - (CH2) n1-; ! d)! where X1 = -OCO- or -COO-, and R2 is H or CH3; ! na is an integer from 0 to 20; preferably n is an integer from 1 to 5; ! n2 is an integer from 0 to 2; ! e)! where Y1 is —CH2 — CH2- (CH2) n2-; or -CH = CH- (CH2) n2-; ! X1, na, n2 and R2 as described above; ! f)! where na and R2 as indicated above, R3 is H or —COCH3; ! with the proviso that when Y is selected from divalent radicals indicated by d) and f), then the —ONO2 group in the formula (I) is bonded to - (CH2) na-; ! provided that