Ripor2 is involved in auditory hair cell stereociliary bundle structure and orientation

Oscar Diaz-Horta, Clemer Abad, Filiz Basak Cengiz, Guney Bademci, Pat Blackwelder, Katherina Walz, Mustafa Tekin

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


RIPOR2 (previously known as FAM65B) localizes to stereocilia of auditory hair cells and causes deafness when its function is disturbed by mutations. Here, we demonstrate that during the morphogenesis of the hair cell bundle, absence of Ripor2 affects the orientation of this key subcellular structure. We show that Ripor2 interacts with Myh9, a protein encoded by a known deafness gene. Absence of Ripor2 is associated with low Myh9 abundance in the mouse cochlea despite increased amount of Myh9 transcripts. While Myh9 is mainly expressed in stereocilia, a phosphorylated form of Myh9 is particularly enriched in the kinocilium. In Ripor2-deficient mice, kinocilium shows an aberrant localization which associates with a reduced content of phosphorylated Myh9. Acetylated alpha tubulin, another specific kinociliary protein which contributes to microtubule stabilization, is reduced in the absence of Ripor2 as well. We propose that Ripor2 deficiency influences abundance and/or post-translational modifications of proteins expressed in both stereocilia and kinocilia. This effect may have a negative impact on the structure and function of the auditory hair cell bundle.

Original languageEnglish (US)
Pages (from-to)1227-1238
Number of pages12
JournalJournal of Molecular Medicine
Issue number11
StatePublished - Nov 1 2018


  • Deafness
  • Hair cells
  • Kinocilium
  • Myh9
  • Ripor2
  • Stereocilia

ASJC Scopus subject areas

  • Molecular Medicine
  • Drug Discovery
  • Genetics(clinical)


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