A number of familial ALS-associated proteins are known to be involved in the proteostasis network, including C9orf72, VCP, p62/sequestosome-1, ubiquilin-2, optineurin, dynactin, and TANK binding kinase 1 (TBK1) (Table 1 and Figure 1). The gene discussed is SQSTM1; the disease is amyotrophic lateral sclerosis.