Transgenic mice were produced to express either wild-type or either of two ALS-linked mutant human FUS broadly throughout the central nervous system (CNS) using the murine prion promoter (Arnold et al., 2013) previously reported to drive transgene expression most abundantly in the CNS, including neurons, astrocytes and oligodendrocytes (see below). The gene discussed is FUS; the disease is amyotrophic lateral sclerosis.