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Phenotypes Associated with This Genotype
Genotype
MGI:5432139
Allelic
Composition		 Clrn1tm2.1Kuna/Clrn1tm2.1Kuna
Genetic
Background		 involves: 129 * BALB/cJ * C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Clrn1tm2.1Kuna mutation (0 available); any Clrn1 mutation (16 available)
♀ phenotype observed in females
♂ phenotype observed in males
N normal phenotype
hearing/vestibular/ear
abnormal cochlear hair cell stereociliary bundle morphology ( J:186316 )
• disrupted integrity that is not as profound at P18 or P21 as in Clrn1tm1.1Kuna homozygotes
• however, tip links are normal
increased or absent threshold for auditory brainstem response ( J:186316 )
• not as profound at P18 or P21 as in Clrn1tm1.1Kuna homozygotes
deafness ( J:186316 )
• early onset hearing loss that progresses to deafness by weaning

nervous system
abnormal cochlear hair cell stereociliary bundle morphology ( J:186316 )
• disrupted integrity that is not as profound at P18 or P21 as in Clrn1tm1.1Kuna homozygotes
• however, tip links are normal

Mouse Models of Human Disease
 DO ID OMIM ID(s) Ref(s)
Usher syndrome type 3A DOID:0110841 OMIM:276902
 J:186316

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
04/23/2024
MGI 6.23 		The Jackson Laboratory