Estrogen-Related Receptor β/NR3B2 Controls Epithelial Cell Fate and Endolymph Production by the Stria Vascularis

Jichao Chen, Jeremy Nathans

Research output: Contribution to journalArticlepeer-review

Abstract

In the mammalian inner ear, endolymph is produced and resorbed by a complex series of epithelia. We show here that estrogen-related receptor β (ERR-β; 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.

Original languageEnglish (US)
Pages (from-to)325-337
Number of pages13
JournalDevelopmental Cell
Volume13
Issue number3
DOIs
StatePublished - Sep 4 2007

Keywords

  • DEVBIO

ASJC Scopus subject areas

  • Molecular Biology
  • Biochemistry, Genetics and Molecular Biology(all)
  • Developmental Biology
  • Cell Biology

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