TDP-43 also regulates processing of Gemin6, a component of the Gem protein complex dysregulated in spinal muscular atrophy (Cauchi, 2014), and of CSNK1D, encoding one of the Ser/Thr Kinase CK-1 isoforms, which can phosphorylate up to 29 sites in TDP-43, some of these in response to stress (Kametani et al., 2009). Here, GEM is linked to proximal spinal muscular atrophy.