Heterogeneous nuclear ribonucleoprotein A1 is the best-known member of the hnRNPs family that has an important role in RNA metabolism and, by playing key roles in neuronal functioning and its depletion, is involved in several neurodegenerative disorders including AD; ALS; spinal muscular atrophy and fronto-temporal lobar degeneration [72]. This evidence concerns the gene HNRNPA1 and proximal spinal muscular atrophy.