First Author | Chen J | Year | 2007 |
Journal | Dev Cell | Volume | 13 |
Issue | 3 | Pages | 325-37 |
PubMed ID | 17765677 | Mgi Jnum | J:124941 |
Mgi Id | MGI:3722975 | Doi | 10.1016/j.devcel.2007.07.011 |
Citation | Chen J, et al. (2007) Estrogen-Related Receptor beta/NR3B2 Controls Epithelial Cell Fate and Endolymph Production by the Stria Vascularis. Dev Cell 13(3):325-37 |
abstractText | 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. |