Most other mouse models of NCL, including the Cln5−/− and Cln6nclf mouse, which mimics late-infantile NCL, display prominent retinal degeneration and microglia reactivity at younger ages (Bartsch et al., 2013; Leinonen et al., 2017; Mirza, 2013). The gene discussed is CLN5; the disease is neuronal ceroid lipofuscinosis.