Both severe and mild SMA mouse models exhibit impaired NMJ maturation markers, including plaque-like morphology of acetylcholine receptor (AChR) clusters, neurofilament (NF) varicosities, and poor terminal arborization (Kong et al., 2009; Martinez et al., 2012; Monani et al., 2003; Kariya et al., 2008). The gene discussed is NFASC; the disease is proximal spinal muscular atrophy.