Loss of Cep290, a putative protein component of Y-links, causes microtubule destabilization in Spata7 mutant photoreceptors. (a) Immunofluorescence images displaying localization of CEP290 (green) throughout CC marked by acetylated α-tubulin (red) in WT and reduction of localization signal to the PCC region in the Spata7 mutant background. Bars: (main images) 5 µm; (single-cilium images) 1 µm. (b) Box plots display the IQR of CEP290 localization signal length quantified for 20 cilia/protein in WT and Spata7 mutant retina (*, P < 0.0001). The whiskers extend to data points that were >1.5× IQR away from first/third quartiles. (c) Representative 2D STORM reconstruction of the CC displaying CEP290 (green) and acetylated α-tubulin (red) throughout the WT CC (left) and at the PCC region in the Spata7 mutant retina (right) with localization length evident from the fluorophore intensity plots on the right. Bars, 200 nm. (d) Hematoxylin and eosin (H&E) staining of paraffin-embedded retinal sections from Spata7^+/−, Spata7^−/−, and Spata7^−/−;Cep290^rd16/+ background assessed at P20 display severe loss of photoreceptor nuclei (outer nuclear layer [ONL]) on a Spata7^−/−;Cep290^rd16/+ background. Bars, 20 µm. INL, inner nuclear layer.