The orphan nuclear hormone receptor ERR-beta controls rod photoreceptor survival.

ABSTRACT: Mutation of rod photoreceptor-enriched transcription factors is a major cause of inherited blindness. We identified the orphan nuclear hormone receptor ERRβ as selectively expressed in rod photoreceptors. Overexpression of ERRβ induces expression of rod-specific genes in retinas of both wildtype and in Nrl-/- mice, which lack rod photoreceptors. Mutation of ERRβ results in dysfunction and degeneration of rods, while inverse agonists of ERRβ trigger rapid rod degeneration, which is rescued by constitutively active mutants of ERRβ. ERRβ coordinates expression of multiple genes that are rate-limiting regulators of ATP generation and consumption in photoreceptors. Furthermore, enhancing ERRβ activity rescues photoreceptor defects that result from loss of the photoreceptor-specific tra

ORGANISM(S): Mus musculus

SUBMITTER: Akishi Onishi 

PROVIDER: E-GEOD-21944 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress