1. Characteristic 1: a pharmaceutical component composed of a formula compound (1)；e. R1 is hydrogen or c8332131 or 832727；R² is hydrogen, cyano, C₁₋₇ alkyl,a. Halogenated tar c83218d8331277o cyclo c832318328；R³ is hydrogen, C₁₋₇ alkyl,or C₃₋₈ cycloalkyl；A is n-heterosexual or n-1-3,N-isocyclopropyl compounds are composed of one or two nitrogen ring atoms, which can be replaced by one, two, three or four alternatives selected by r83088；R 2 is an isocyclic aryl group consisting of one nitrogen ring atom, which can be replaced by one, two, three or four alternatives selected by r83088；R¹³ is hydrogen, C₁₋₇ alkyl or C₃₋₈ cycloalkyl；R¹⁴ is independently selected from nitrogen, C₁₋₇ alkyl,amino, amino C₁₋₇ alkyl,C₃₋₈ cycloalkyl and heterocycloalkyl or two R¹⁴ together form C₁₋₇ alkylene；with the proviso that if A is a UN-heterocycle alkyl comprising only 1 nitrogen ring atom, then at least one R sustitu substituent is amino or C₁₋₇-amino-alkyl；Or a pharmaceutically acceptable salt； the ingredient is an oral water solution or a dry powder suitable for oral water solution. Claim 21: the pharmaceutical ingredients in claims 1 to 20 include: 7 - (4.7-diazo [2.5] octan-7-il) - 2 - (2.8-dimethylpyridine [1.2-b] pyridine-6-il) pyridine [1.2-A] pyridine-4 -, or an acceptable pharmaceutical salt, accounting for 1% to 10% by weight； 5% to 15% by weight. 1. A tampon system selected between citric acid, maleic acid, maleate or tar；By weight, the diluent content selected from Manitoba or the mixture of Manitoba and imarter is 40-70%； by weight of fatty acid, it is 1-4%； by weight of deoxyphenylacetic acid, it is 0.5-2%； by weight of PEG6000, it is 0.5-2%； by weight of sodium or sodium benzoate, it is 1-8%； 0% to 3% by weight for sweeteners extracted from soda sugar or sodium, 0% to 20% by weight for fragrances selected from strawberry or vanilla, and no more than 100% by weight for total ingredients. Requirement 46: combine a cutting modulator with SMN2 gene connection and a citoprot
