Estrogen-related receptor beta/NR3B2 controls epithelial cell fate and endolymph production by the stria vascularis

Dev Cell. 2007 Sep;13(3):325-37. doi: 10.1016/j.devcel.2007.07.011.


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.

Publication types

  • Comparative Study

MeSH terms

  • Alleles
  • Animals
  • Cell Differentiation
  • Deafness / etiology
  • Deafness / pathology
  • Ear, Inner / cytology
  • Ear, Inner / metabolism
  • Endolymph / metabolism*
  • Epithelial Cells / metabolism*
  • Epithelial Cells / physiology
  • Estrogen Receptor beta / metabolism*
  • Gene Expression
  • Immunohistochemistry
  • In Situ Hybridization
  • Mice
  • Mice, Knockout
  • Models, Biological
  • Mosaicism
  • Stria Vascularis / cytology
  • Stria Vascularis / metabolism*


  • Estrogen Receptor beta

Associated data

  • GEO/GSE8434