Journal of Neurocytology

, Volume 32, Issue 9, pp 1143–1154

The homeobox gene Emx2 underlies middle ear and inner ear defects in the deaf mouse mutant pardon

Authors

  • Charlotte R. Rhodes
    • MRC Institute of Hearing ResearchUniversity Park
  • Nicholas Parkinson
    • MRC Institute of Hearing ResearchUniversity Park
  • Hsun Tsai
    • MRC Institute of Hearing ResearchUniversity Park
  • Debra Brooker
    • MRC Institute of Hearing ResearchUniversity Park
  • Siobhan Mansell
    • MRC Institute of Hearing ResearchUniversity Park
  • Nigel Spurr
    • GlaxoSmithKline Pharmaceuticals
  • A. Jackie Hunter
    • GlaxoSmithKline Pharmaceuticals
    • MRC Institute of Hearing ResearchUniversity Park
  • Steve D. M. Brown
    • MRC Institute of Hearing ResearchUniversity Park
Article

DOI: 10.1023/B:NEUR.0000021908.98337.91

Cite this article as:
Rhodes, C.R., Parkinson, N., Tsai, H. et al. J Neurocytol (2003) 32: 1143. doi:10.1023/B:NEUR.0000021908.98337.91
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Abstract

The semi-dominantly inherited mouse mutation pardon (Pdo) was isolated due to the lack of a Preyer reflex (ear flick) in response to sound from a large-scale N-ethyl-N-nitrosourea (ENU) mutagenesis programme. Dissection of the middle ear revealed malformations in all three ossicles, rendering the ossicular chain incomplete. Hair cell counts in the apical turn of the organ of Corti revealed a significant 22.7% increase in the number of outer hair cells. Raised compound action potential thresholds in Pdo/+ mutants suggested a combined sensorineural/conductive hearing loss. We show that a missense mutation in the homeobox gene Emx2 is responsible for these defects, identifying a new function for this gene in the development of specific structures in the ear.

Copyright information

© Kluwer Academic Publishers 2003