STUB1 and Brain atrophy: We found that these models recapitulate the key features of SCAR16, notably: decreased steady-state protein expression due to proteasome-dependent turnover (Figs 4E, 4F, 4G, 5A, 5B, 5F and 5G); brain atrophy (Fig 6D); increased mortality (Fig 6A); loss of motor function (Fig 7A and 7F); age-dependent impairment of sensorimotor function (Fig 8B); overt hyperactivity (Fig 8C, 8D and 8E); and impaired cognitive function (Fig 9A, 9B, 9C and 9D).