FIELD: chemistry.
SUBSTANCE: invention relates to use of a composition containing at least 20-hydroxyecdysone and/or at least one semisynthetic derivative of 20-hydroxyecdysone, for the treatment of a specific disorder of motor neurons in mammals suffering from a neuromuscular disease, including a change in muscle function due to a specific disorder of motor neurons, wherein said disease is spinal muscular atrophy (SMA) and said semisynthetic derivative of 20-hydroxyecdysone is selected from a compound of formula (I) or a compound of general formula (II). In general formula (I) R1 is selected from: (C1-C6)W(C1-C6) group; (C1-C6)W(C1-C6)W(C1-C6) group; (C1-C6)W(C1-C6)CO2(C1-C6) group; (C1-C6)A group, wherein A is a heterocycle optionally substituted with a group selected from the type OH, OMe, (C1-C6), N(C1-C6), CO2(C1-C6), group CH2Br; W is a heteroatom selected from N, O and S, preferably O, but more preferably S.
EFFECT: using the invention enables treating spinal muscular atrophy (SMA) effectively.
14 cl, 13 dwg
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