Fig. 9.
Defective mitochondria and ERG response in Nrf1f/f;Rho-iCre retina. (a, b) Transmission electron microscopy (TEM) images of the inner segments of wildtype and Nrf1f/f;Rho-iCre photoreceptors. Inner segment is color-labeled in red, and mitochondria are circled in blue. Asterisks indicate clustered mitochondria near the OLM in Nrf1f/f;Rho-iCre ISs. Insets show higher magnification images of indicated areas. (c–e) TEM images were analyzed with Fiji for the circularity of ISs (c), size of ISs (d) and the number of mitochondria (e). f, g TEM images of the outer segments of wildtype (f) and Nrf1f/f;Rho-iCre (g) photoreceptors. (h, i) Immunostaining of 6-week wildtype (h) and Nrf1f/f;Rho-iCre (i) retinal sections with anti-Tfam antibody. Arrowheads indicate Tfam staining in ISs. (j, k) COX activity of 7-week wildtype (j) and Nrf1f/f;Rho-iCre (k) retinal sections. Arrowheads indicate COX activities in ISs. (l) qRT-PCR analysis of genes involved in mitochondria fusion of wildtype and Nrf1f/f;Rho-iCre retinas. (m) Mitochondria copy number of 6-week-old wildtype and Nrf1f/f;Rho-iCre retinas. (n, o) ERGs of wildtype and Nrf1f/f;Rho-iCre littermates under dark-adapted (scotopic, n) and light-adapted (photopic, o) conditions. Scale bars: 1 μm in a, b, f and g, 10 μm in h–k. OLM: outer limiting membrane. OS: outer segment. IS: inner segment. ONL: outer nuclear layer. WT: wildtype