Figure 4

Dysregulation of lysosomal proteins in PPT1-deficient retinas. Expression levels of LAMP1, LAMP2, CTSD and CTSZ were slightly elevated in 45-day-old mutant animals (b,f,j,n, respectively) when compared to age-matched wild-type mice (a,e,i,m, respectively). Pronounced upregulation of LAMP1-, LAMP2-, CTSD- and CTSZ-immunoreactivity was observed in 240-day-old Ppt1 ko retinas (d,h,l,p, respectively). The corresponding immunostainings of P240 control retinas are shown in (c,g,k,o), respectively. gcl: ganglion cell layer; inl: inner nuclear layer; ipl: inner plexiform layer; onl: outer nuclear layer. Scale bar in (p) for (aāp): 100āµm.