The study of neuronal RNA granules have driven considerable attention since the discovery that the RNA-binding proteins FMRP, TDP-43, and SMN, respectively associated with Fragile X Syndrome [80], amyotrophic lateral sclerosis [81,82] and spinal muscular atrophy [83,84], are components of RNA granules. Here, TARDBP is linked to proximal spinal muscular atrophy.