SOD1H46R-tg mouse recapitulates many neurological and pathological features observed not in only familial but also in sporadic ALS patients, which include progressive motor neuron degeneration, impaired motor control, and accumulation of SQSTM1 and ubiquitin-positive aggregates in the spinal cord [19, 20, 22, 23]. The gene discussed is SQSTM1; the disease is amyotrophic lateral sclerosis.