C57BL/6 mice develop severe and progressive late-onset hearing loss. Histopathological changes associated with age-related hearing loss includes the disruption of both outer and inner hair cells. C57BL/6 mice are also more susceptible to noise-induced hearing loss. Age related hearing loss 1 Cdh23 (formerly Ahl), a major gene responsible for this hearing loss, was mapped in an intersubspecific backcross by measuring elevated auditory-evoked brainstem response (ABR) thresholds. Cdh23 is located on Chromosome 10 near marker D10Mit5. B6.CAST-Cdh23Ahl+ is a congenic strain that carries the wildtype allele of Cdh23 producing C57BL/6 mice that are not susceptible to age related hearing loss.
The normal functioning Cdh23 allele from the CAST/EiJ strain was backcrossed onto the C57BL/6J background for 10 backcross generations and then the strain was sibling intercrossed to homozygosity. This strain reached generation N10F19 in 2009.
|Allele Name||wild-type of age related hearing loss|
|Allele Type||Not Applicable|
|Allele Synonym(s)||Cdh23753G; Cdh23CAST; Cdh23mdfw-ahl-CAST/Ei|
|Gene Symbol and Name||Cdh23, cadherin 23 (otocadherin)|
|Strain of Origin||CAST/EiJ|
|Molecular Note||This variant of the SNP rs257098870 allele contrasts with the variant of the allele found in C57BL/6J in that a G is present at coding nucleotide position 753 rather than an A. Thus, the G-GT splice donor site is maintained.|
When using the B6.CAST-Cdh23Ahl+/Kjn mouse strain in a publication, please cite the originating article(s) and include JAX stock #002756 in your Materials and Methods section.