Figure 2

Semithin sections and electron micrographs of the P65 Npc1 ^+/+and Npc1 ^-/-retina. A, B: Semithin sections; A shows regular retinal formation with internal plexiform layer (IPL), ganglion cell layer (GC), nerve fiber layer (NFL) and inner limiting (glia) membrane (ILM); B: In the Npc1^-/- retina, a large ganglion cell (asterisk) and smaller dark stained extensions of a Müller cell (arrow) contain deposits; C-F: Transmission electron microscopy of the inner layers of Npc1^+/+ retina (C) and Npc1^-/- retina (D). The nucleus marked with an asterisk in C is either an astrocyte or an “untypical Müller cell” located on the periphery of the fovea [46]. The organization of Müller cell endfeet seems disturbed in Npc1^-/- retina (D). Dark cytoplasmic extensions of Müller cells contain autophagosomes (** in D) and the same is visible in somata of a ganglion cell (GC; * in D). E shows a representative ganglion cell containing many large myelin-like deposits in phagolysosomes. The rectangle refers to a magnification in F. The arrow points to myelin deposits within a membrane-bound compartment. Abbreviations: Ca, capillary; MF, Müller cell endfeet, NFL, nerve fiber layer. Scale bars: 10 μm in A, B; 5 μm in C, D; 2.5 μm in E; 250 nm in F.