Notes: RG3039 is an inhibitor of mRNA decapping scavenger enzyme (DcpS; IC50 = 0.069 nM).{65258} It prevents denervation of the neuromuscular junction (NMJ) and decreases in NMJ synaptic transmission and myofiber size of the longissimus muscle in a mouse model of spinal muscular atrophy (SMA) induced by knockout of the gene encoding survival motor neuron 1 (Smn1) when administered at a dose of 10 mg/kg.{65259} RG3039 improves survival and motor function in the same model.