Journal article
The Coxsackievirus and Adenovirus Receptor: A new adhesion protein in cochlear development
Hearing research, Vol.215(1), pp.1-9
2006
DOI: 10.1016/j.heares.2006.02.009
PMID: 16678988
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.
Details
- Title: Subtitle
- The Coxsackievirus and Adenovirus Receptor: A new adhesion protein in cochlear development
- Creators
- Katherine J.D.A Excoffon - Department of Internal Medicine, Division of Pulmonary Medicine, University of Iowa, 440 EMRB, Iowa City, IA 52242, USAMatthew R Avenarius - Department of Otolaryngology, Head and Neck Surgery, University of Iowa, Iowa City, IA 52242, USAMarlan R Hansen - Department of Otolaryngology, Head and Neck Surgery, University of Iowa, Iowa City, IA 52242, USAWilliam J Kimberling - Center for Hereditary Communication Disorders, Boys Town National Research Hospital, Omaha, NE 68131, USAHossein Najmabadi - Genetics Research Center, The Social Welfare and Rehabilitation Sciences University, Tehran, IranRichard J.H Smith - Department of Otolaryngology, Head and Neck Surgery, University of Iowa, Iowa City, IA 52242, USAJoseph Zabner - Department of Internal Medicine, Division of Pulmonary Medicine, University of Iowa, 440 EMRB, Iowa City, IA 52242, USA
- Resource Type
- Journal article
- Publication Details
- Hearing research, Vol.215(1), pp.1-9
- DOI
- 10.1016/j.heares.2006.02.009
- PMID
- 16678988
- NLM abbreviation
- Hear Res
- ISSN
- 0378-5955
- eISSN
- 1878-5891
- Publisher
- Elsevier B.V
- Language
- English
- Date published
- 2006
- Academic Unit
- Roy J. Carver Department of Biomedical Engineering; Molecular Physiology and Biophysics; Pulmonary, Critical Care, and Occupational Medicine; Anatomy and Cell Biology; Stead Family Department of Pediatrics; Iowa Neuroscience Institute; Neurosurgery; Otolaryngology; Internal Medicine
- Record Identifier
- 9984006484802771
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