title: Estrogen-related receptor beta/NR3B2 controls epithelial cell fate and endolymph production by the stria vascularis.
abstract: In the mammalian inner ear, endolymph is produced and resorbed by a complex series of epithelia. We show here that estrogen-related receptor beta (ERR-beta; NR3B2), an orphan nuclear receptor, is specifically expressed in and controls the development of the endolymph-producing cells of the inner ear: the strial marginal cells in the cochlea and the vestibular dark cells in the ampulla and utricle. Nr3b2(-/-) strial marginal cells fail to express multiple ion channel and transporter genes, and they show a partial transformation toward the fate of the immediately adjacent Pendrin-expressing epithelial cells. In genetically mosaic mice, Nr3b2(-/-) strial marginal cells produce secondary alterations in gene expression in the underlying intermediate cells and a local loss of strial capillaries. A systematic comparison of transcripts in the WT versus Nr3b2(-/-) stria vascularis has identified a set of genes that is likely to play a role in the development and/or function of endolymph-producing epithelia.
authors.affiliation: Department of Molecular Biology and Genetics, Johns Hopkins University School of Medicine, Baltimore, MD 21205, USA.
authors.forename: Jichao
authors.initials: J
authors.lastname: Chen
authors.forename: Jeremy
authors.initials: J
authors.lastname: Nathans
mesh_terms: D000483:Alleles; D000818:Animals; D002454:Cell Differentiation; D003638:Deafness / Q000209:etiology / Q000473:pathology; D007758:Ear, Inner / Q000166:cytology / Q000378:metabolism; D004710:Endolymph / Q000378:metabolism*; D004847:Epithelial Cells / Q000378:metabolism* / Q000502:physiology; D047629:Estrogen Receptor beta / Q000378:metabolism*; D015870:Gene Expression; D007150:Immunohistochemistry; D017403:In Situ Hybridization; D051379:Mice; D018345:Mice, Knockout; D008954:Models, Biological; D009030:Mosaicism; D013316:Stria Vascularis / Q000166:cytology / Q000378:metabolism*
chemical_list: D047629:Estrogen Receptor beta