Similar defects in inversin/NPHP-2 distribution were also detected in CFH knockout mice and human photoreceptors from individuals homozygous for the AMD high-risk CFH Y402H variant, suggesting that cilia structural defects make a significant, yet unappreciated contribution to the progressive photoreceptor dysfunction observed in AMD patients with CFH loss-of-function mutations (15). Here, INVS is linked to age-related macular degeneration.