Retinoid isomerase inhibitors impair but do not block mammalian cone photoreceptor function.

ABSTRACT: Visual function in vertebrates critically depends on the continuous regeneration of visual pigments in rod and cone photoreceptors. RPE65 is a well-established retinoid isomerase in the pigment epithelium that regenerates rhodopsin during the rod visual cycle; however, its contribution to the regeneration of cone pigments remains obscure. In this study, we use potent and selective RPE65 inhibitors in rod- and cone-dominant animal models to discern the role of this enzyme in cone-mediated vision. We confirm that retinylamine and emixustat-family compounds selectively inhibit RPE65 over DES1, the putative retinoid isomerase of the intraretinal visual cycle. In vivo and ex vivo electroretinography experiments in Gnat1^-/- mice demonstrate that acute administration of RPE65 in

SUBMITTER: Kiser PD 

PROVIDER: S-EPMC5881442 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature