However, studies on proximal spinal muscular atrophy (SMA) indicate that nonsense suppression (induced by treatment with aminoglycosides) of alternatively spliced survival motor neuron 2 (SMN2) transcripts leads to the extension and stabilization of the SMN protein, elevated SMN protein levels and improved motor function in SMA mice in vivo [47,48,49,50]. The gene discussed is SMN1; the disease is proximal spinal muscular atrophy.