The vertebrate eye contains numerous ciliated cell types, the most well known are the light sensitive photoreceptors in the outer retina, which contain a highly specialised primary cilium, the connecting cilium and outer segment. It is well established that defective ciliary trafficking in the photoreceptor is likely the predominant cause of retinal degeneration in ciliopathies. However defects in many other ciliated ocular cell types likely contribute to a vast number of syndromic and non-syndromic ocular diseases. Of particular interest is the retinal pigment epithelium (RPE), a ciliated monolayer epithelium, which supports the retina and is essential for visual transduction. We have previously shown that the primary cilium is required for regulation of WNT signaling during maturation of the RPE and likely contributes to progression of visual decline. We have established several different cilia mutant mouse models that recapitulate the patient phenotype. In this project we will manipulate ciliary WNT signalling during RPE development to improve RPE maturation. We will test pharmacologically active compounds in vivo and in vitro in an effort to counteract the effect of disrupted ciliary WNT signalling in the RPE and ultimately reduce the rate of retinal degeneration.