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Association of cadherin 23 with polygenic inheritance and genetic modification of sensorineural hearing loss

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Abstract

Age-related hearing loss (AHL) in common inbred mouse strains is a genetically complex quantitative trait. We found a synonymous single-nucleotide polymorphism in exon 7 of Cdh23 that shows significant association with AHL and the deafness modifier mdfw (modifer of deafwaddler). The hypomorphic Cdh23753A allele causes in-frame skipping of exon 7. Altered adhesion or reduced stability of CDH23 may confer susceptibility to AHL. Homozygosity at Cdh23753A or in combination with heterogeneous secondary factors is a primary determinant of AHL in mice.

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Figure 1: Positional cloning of ahl and mdfw.
Figure 2: Cdh23753A affects splicing of exon 7.

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Acknowledgements

We thank R. Pellegrino, M. Irby, A. Calderon, A. Derr and J. Fiallos for technical assistance, K. Steel for the V/Le strain and C. Kubisch, N. Fischel-Ghodsian and D. Drayna for discussions and their comments on the manuscript.

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Authors and Affiliations

  1. Section on Neurogenetics, National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Rockville, 20850, Maryland, USA

    Konrad Noben-Trauth

  2. The Jackson Laboratory, 600 Main Street, Bar Harbor, 04609, Maine, USA

    Qing Yin Zheng & Kenneth R Johnson

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  1. Konrad Noben-Trauth
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  2. Qing Yin Zheng
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  3. Kenneth R Johnson
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Correspondence to Konrad Noben-Trauth.

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Noben-Trauth, K., Zheng, Q. & Johnson, K. Association of cadherin 23 with polygenic inheritance and genetic modification of sensorineural hearing loss. Nat Genet 35, 21–23 (2003). https://doi.org/10.1038/ng1226

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