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Phenotypes associated with this allele
Allele Symbol
Allele Name
Allele ID 		Cdh23tm1.1Kjn
targeted mutation 1.1, Kenneth R Johnson
MGI:5897899
Summary 2 genotypes


Genotype
MGI:5898244
hm1
Allelic
Composition		 Cdh23tm1.1Kjn/Cdh23tm1.1Kjn
Genetic
Background		 129S/Sv-Cdh23tm1.1Kjn/Kjn
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23tm1.1Kjn mutation (1 available); any Cdh23 mutation (281 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
hearing/vestibular/ear
decreased cochlear hair cell number ( J:241137 )
• at 6 months of age there is loss of both inner and outer hair cells that is greater than that seen in 9 month old 129S1/SvImJ controls
increased or absent threshold for auditory brainstem response ( J:241137 )
• Background Sensitivity: relative to 129S1/SvImJ controls homozygotes have elevated ABR thresholds from one month of age on at 16 kHz and 32 kHz, with 32 kHz response nearly absent at this age, and elevated ABR at 8 kHz but less severe than the higher frequency loss

nervous system
decreased cochlear hair cell number ( J:241137 )
• at 6 months of age there is loss of both inner and outer hair cells that is greater than that seen in 9 month old 129S1/SvImJ controls




Genotype
MGI:5898375
cx2
Allelic
Composition		 Cdh23ahl/Cdh23tm1.1Kjn
MahlC57BL/6J/Mahl129S1/SvImJ
Genetic
Background		 (129S1.B6-(rs3696307-rs257098870)/Kjn x 129S/Sv-Cdh23tm1.1Kjn/Kjn)F1
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Cdh23ahl mutation (55 available); any Cdh23 mutation (281 available)
Cdh23tm1.1Kjn mutation (1 available); any Cdh23 mutation (281 available)
Mahl129S1/SvImJ mutation (1 available); any Mahl mutation (2 available)
MahlC57BL/6J mutation (1 available); any Mahl mutation (2 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
hearing/vestibular/ear
increased or absent threshold for auditory brainstem response ( J:241137 )
• these mice, homozygous for the c.735A point mutation that causes age related hearing loss and heterozgyous for the ameliorating C57BL/6J-derived modifier, have a 16 kHz ABR threshold at 3 months of age that is less than mice homozygous for the 129S1/SvImJ-derived modifier, proving the C57BL/6J allele dominant




Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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Send questions and comments to User Support. 		last database update
12/10/2024
MGI 6.24 		The Jackson Laboratory