On the other hand, mutations in proteins that participate physiologically in the RNA metabolism, including TDP-43 and FUS (fusion involved in malignant liposarcoma; Kwiatkowski et al., 2009; Vance et al., 2009) were described in ALS patients suggesting the involvement of an aberrant RNA-metabolism in the disease etiology (Sreedharan et al., 2008; Kwiatkowski et al., 2009; DeJesus-Hernandez et al., 2011; Renton et al., 2011). The gene discussed is TARDBP; the disease is amyotrophic lateral sclerosis.