The Coxsackievirus and Adenovirus Receptor: A new adhesion protein in cochlear development

Katherine J.D.A. Excoffon, Matthew R. Avenarius, Marlan R. Hansen, William J. Kimberling, Hossein Najmabadi, Richard J.H. Smith, Joseph Zabner

    Research output: Contribution to journalArticlepeer-review

    25 Scopus citations

    Abstract

    The Coxsackievirus and Adenovirus Receptor (CAR) is an essential regulator of cell growth and adhesion during development. The gene for CAR, CXADR, is located within the genomic locus for Usher syndrome type 1E (USH1E). Based on this and a physical interaction with harmonin, the protein responsible for USH1C, we hypothesized that CAR may be involved in cochlear development and that mutations in CXADR may be responsible for USH1E. The expression of CAR in the cochlea was determined by PCR and immunofluorescence microscopy. We found that CAR expression is highly regulated during development. In neonatal mice, CAR is localized to the junctions of most cochlear cell types but is restricted to the supporting and strial cells in adult cochlea. A screen of two populations consisting of non-syndromic deaf and Usher 1 patients for mutations in CXADR revealed one haploid mutation (P356S). Cell surface expression, viral receptor activity, and localization of the mutant form of CAR were indistinguishable from wild-type CAR. Although we were unable to confirm a role for CAR in autosomal recessive, non-syndromic deafness, or Usher syndrome type 1, based on its regulation, localization, and molecular interactions, CAR remains an attractive candidate for genetic deafness.

    Original languageEnglish (US)
    Pages (from-to)1-9
    Number of pages9
    JournalHearing Research
    Volume215
    Issue number1-2
    DOIs
    StatePublished - May 2006

    Keywords

    • CAR
    • CXADR
    • Cochlea
    • Coxsackievirus and Adenovirus Receptor
    • Deafness
    • Usher syndrome

    ASJC Scopus subject areas

    • Sensory Systems

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