Ciliary proteins bbs8 and ift20 promote planar cell polarity in the cochlea

Helen L. May-Simera, Ronald S. Petralia, Mireille Montcouquiol, Ya Xian Wang, Katherine B. Szarama, Yun Liu, Weichun Lin, Michael R. Deans, Gregory J. Pazour, Matthew W. Kelley

Research output: Contribution to journalArticlepeer-review

54 Scopus citations


Primary cilia have been implicated in the generation of planar cell polarity (PCP). However, variations in the severity of polarity defects in different cilia mutants, coupled with recent demonstrations of non-cilia-related actions of some cilia genes, make it difficult to determine the basis of these polarity defects. To address this issue, we evaluated PCP defects in cochlea from a selection of mice with mutations in cilia-related genes. Results indicated notable PCP defects, including mis-oriented hair cell stereociliary bundles, in Bbs8 and Ift20 single mutants that are more severe than in other cilia gene knockouts. In addition, deletion of either Bbs8 or Ift20 results in disruptions in asymmetric accumulation of the core PCP molecule Vangl2 in cochlear cells, suggesting a role for Bbs8 and/or Ift20, possibly upstream of core PCP asymmetry. Consistent with this, co-immunoprecipitation experiments indicate direct interactions of Bbs8 and Ift20 with Vangl2. We observed localization of Bbs and Ift proteins to filamentous action as well as microtubules. This could implicate these molecules in selective trafficking of membrane proteins upstream of cytoskeletal reorganization, and identifies new roles for cilia-related proteins in cochlear PCP.

Original languageEnglish (US)
Pages (from-to)555-566
Number of pages12
JournalDevelopment (Cambridge)
Issue number3
StatePublished - Feb 1 2015


  • Actin
  • Cilia
  • Cochlea
  • Microtubules
  • Mouse
  • Polarity

ASJC Scopus subject areas

  • Molecular Biology
  • Developmental Biology


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