Fig. 9.

The pathological changes of RPE in NFE2L2/PGC-1α dKO mice. The representative confocal microscopic images of one-year old (A–D) and (H–K) dKO samples indicate various dry AMD resembling signs. The accumulation of intracellular lipofuscin-like material (white arrowhead; H), extracellular ubiquitin (Ubg) positivity (read arrowhead; I), restricted apoptosis (green arrow; J) and outer nuclear layer (ONL) atrophy (K) can be observed in the dKO samples. The TEM images of the WT (E–G) and dKO (L–O) samples. The basal folding of RPE is well-preserved in WT (E,G), while their disruption occurs in dKO (L,O). The thicker-disrupted Bruch's membrane (BM) of the dKO sample is shown in image L (black arrow), where electron-dense amorphous debris (yellow arrrow; O) and the membranous debris (blue arrowhead) are abundant compared to the WT sample (G). Alterations of microvilli structures (blue arrow; E,L), increased number of melanin pigments (red arrowheads; F,M), the damaged photoreceptor layer (E,L), enlarged vacuole-like structures (pink arrow; L and blue arrows; F,M) are more visible in dKO. N means nuclei and E endothelial cell nuclei. The scale bars for A, H = 5 μm, B, I and I–K = 2 μm, C, J = 5 μm, D, K = 2 μm, E,L = 5 μm, F, M and G, O Data represent similar results shown in the Felszeghy et al. (2019) Redox Biology publication.