Figure 2

Accumulation of autofluorescent materials in the thalamus of CtsD-CKO mice. Representative images of lipofuscin autofluorescence in the thalamus of CtsD-CKO mice at p23 is shown in (B), as compared with that in control mice (A). Insets in (B) are higher magnified images of autofluorescence by confocal microscopy using light at 568 and 488 nm for excitation. “Merge” is a merged image of the two (568 and 488). Autofluorescent puncta in the thalamus were quantified. (***p < 0.001, from p19 to p25, n = 3 sections form separate mice) (C). Representative electron microscopic images of control (D) and CtsD-CKO mouse thalami (E–I) at p25 are shown. (E) and (F) show representative images of neurons in CtsD-CKO mouse thalamus, which contains numerous dense bodies, resembling granular osmiophilic deposits (GROD), and vacuolar/autophagosome-like structures (AV). A phagocytic cell possesses a degenerated flatten nucleus (white arrow) (G). (H) and (I) show a spheroid-like axon containing numerous AVs and with thinner myelin. (F) and (I) are higher magnification images of the boxes in respective (E) and (H).