Format

Send to

Choose Destination
Development. 2015 Feb 1;142(3):555-66. doi: 10.1242/dev.113696.

Ciliary proteins Bbs8 and Ift20 promote planar cell polarity in the cochlea.

Author information

1
Section on Developmental Neuroscience, Laboratory of Cochlear Development, National Institute on Deafness and other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA maysimerah@nei.nih.gov.
2
Advanced Imaging Core, National Institute on Deafness and other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA.
3
Planar Polarity and Plasticity Group, Institut National de la Santé et de la Recherche Médicale U862, Neurocenter Magendie, 33077 Bordeaux, France.
4
Section on Developmental Neuroscience, Laboratory of Cochlear Development, National Institute on Deafness and other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA Department of Cell and Molecular Biology, St. Jude Children's Research Hospital, Memphis, TN 38105, USA.
5
Department of Neuroscience, UT Southwestern Medical Center, Dallas, TX 75235, USA.
6
Division of Otolaryngology-Head and Neck Surgery and Department of Neurobiology & Anatomy, University of Utah School of Medicine, Salt Lake City, UT 84132, USA.
7
Program in Molecular Medicine, University of Massachusetts Medical School, Worcester, MA 01605, USA.
8
Section on Developmental Neuroscience, Laboratory of Cochlear Development, National Institute on Deafness and other Communication Disorders, National Institutes of Health, Bethesda, MD 20892, USA.

Abstract

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 actin 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.

KEYWORDS:

Actin; Cilia; Cochlea; Microtubules; Mouse; Polarity

PMID:
25605782
PMCID:
PMC4302998
DOI:
10.1242/dev.113696
[Indexed for MEDLINE]
Free PMC Article

Supplemental Content

Full text links

Icon for HighWire Icon for PubMed Central
Loading ...
Support Center