Although the Hgstn/tn mice represent the first report linking ESCRT-0 to neuromuscular disease, mutations in the ESCRT-III component CHMP2B have been linked to amyotrophic lateral sclerosis [27,28], and patients with CHMP2B mutations exhibit phenotypes consistent with lower motor neuron disease [28]. The gene discussed is CHMP2B; the disease is amyotrophic lateral sclerosis.