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Phenotypes Associated with This Genotype
Genotype
MGI:5314998
Allelic
Composition
Tmc1nice/Tmc1nice
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tmc1nice mutation (0 available); any Tmc1 mutation (29 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• at 4 weeks of age, the inner row of stereocilia of the OHC hair bundle is often irregular in length or missing
• in areas not showing degeneration the innermost row of stereocilia are nonuniform in height and missing many stereocilia
• by 8 weeks of age IHCs are completely absent from the basal region and in the mid region IHC's are largely degenerated and hair bundles are absent
• signs of degeneration are also seen in the apical region at 8 weeks of age
• by 8 weeks of age OHCs are completely absent from the basal region and in the mid region OHC's are largely degenerated and hair bundles are absent
• signs of degeneration are also seen in the apical region at 8 weeks of age
• by 8 weeks of age in the mid region
• degeneration is detected as early as 2 weeks of age
• complete degeneration of the sensory epithelium in the basal and mid region at 4 weeks of age
• signs of degeneration are also seen in the apical region at 8 weeks of age
• profound hearing loss at 4 weeks of age, ranging from 100 to greater than 120 dB SPL

behavior/neurological
N
• no circling or head tossing/tilting behaviors are seen

nervous system
• at 4 weeks of age, the inner row of stereocilia of the OHC hair bundle is often irregular in length or missing
• in areas not showing degeneration the innermost row of stereocilia are nonuniform in height and missing many stereocilia
• by 8 weeks of age IHCs are completely absent from the basal region and in the mid region IHC's are largely degenerated and hair bundles are absent
• signs of degeneration are also seen in the apical region at 8 weeks of age
• by 8 weeks of age OHCs are completely absent from the basal region and in the mid region OHC's are largely degenerated and hair bundles are absent
• signs of degeneration are also seen in the apical region at 8 weeks of age

Mouse Models of Human Disease
OMIM ID Ref(s)
Deafness, Autosomal Recessive 7; DFNB7 600974 J:181985


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
09/27/2016
MGI 6.05
The Jackson Laboratory