PPT1 and CLN5 disease: Nevertheless, our findings in a mouse model of juvenile CLN3 disease complement molecular, structural, and functional studies in various animal models of infantile CLN1 disease (Virmani et al., 2005; Kim et al., 2008; Kielar et al., 2009), late infantile CLN6 disease (Kielar et al., 2009), congenital CLN10 disease (Koch et al., 2011), and late infantile CLN5 disease (Amorim et al., 2015), and suggest that early synaptic alteration is a characteristic feature of NCLs.