The fact that a mutation in DNAJB6 is associated with limb-girdle muscular dystrophy which in turn is accompanied by pathological TDP-43 aggregates, indicates a protective function of DNAJB6 against dysregulated TDP-43 SG formatio (Harms et al., 2012). The gene discussed is DNAJB6; the disease is limb-girdle muscular dystrophy.