Fig. 7.

CLDN mutants induced degeneration of the outer nuclear layer (ONL) and the remodeling of bipolar cells in the inner nuclear layer (INL). The ganglion cell layer (GCL) layer was unaffected. Data were collected 30 days after transduction on postnatal day 0 (PN0). Green fluorescent protein (GFP) revealed transduced cells only in the ONL (b–e). The retinal pigment epithelium (RPE) is not included in the images. a Schema of the retina defines the "a, b, c" labels used in (b, e). b TUNEL (terminal deoxynucleotidyl transferase dUTP nick end labeling) staining indicated that photoreceptor cell death induced by CLDN19^G20D>CLDN19^R81W>CLDN19^WT and was only observed in the ONL. c, d The impression in b was confirmed by quantification of apoptosis and ONL thickness. e Immunofluorescence staining revealed ganglion cell nuclei (BRN3) and the neurites of bipolar cells (protein kinase Cα (PKCα)). f Schematic diagram of a bipolar cell. The bars indicate where measurements were made for axon and dendritic length. g Quantification of axon and dendritic length in the retinal bipolar cell layer. The length of axons and dendrites was reduced in retinas transduced with mutated CLDN19. Measurements were made from four different loci from three mice in each group. IS photoreceptor inner segment, OS photoreceptor outer segment, ONL outer nuclear layer, OPL outer plexiform layer, INL inner nuclear layer, IPL inner plexiform layer, RGC retinal ganglion cell layer. Error bars, SEM (*P < 0.05, **P < 0.01,***P < 0.001). Scale bar, 20 µm