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Phenotypes Associated with This Genotype
Genotype
MGI:5315001
Allelic
Composition
Tmc1baringo/Tmc1baringo
Genetic
Background
involves: C57BL/6
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tmc1baringo mutation (0 available); any Tmc1 mutation (28 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• at 4 weeks of age, the inner (smallest) row of stereocilia is often missing or, if present, is of irregular height
• by 8 weeks of age remaining IHCs in the basal and mid region show severe degeneration and fusion of stereocilia
• at 4 weeks of age, the inner (smallest) row of stereocilia is often missing or, if present, is of irregular height
• by 8 weeks of age lateral supporting cells show irregular morphological characteristics
• at 4 weeks of age extensive degeneration of hair and supporting cells, and the collapse of the tunnel of Corti, is evident in the basal to mid region
• severe to profound hearing loss at 4 weeks of age, ranging from 85 to 120 dB SPL

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

nervous system
• at 4 weeks of age, the inner (smallest) row of stereocilia is often missing or, if present, is of irregular height
• by 8 weeks of age remaining IHCs in the basal and mid region show severe degeneration and fusion of stereocilia
• at 4 weeks of age, the inner (smallest) row of stereocilia is often missing or, if present, is of irregular height

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


Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
04/26/2016
MGI 6.03
The Jackson Laboratory