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Phenotypes Associated with This Genotype
Genotype
MGI:2177312
Allelic
Composition
Tmc1dn/Tmc1dn
Genetic
Background
STOCK Grhl3ct/J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Tmc1dn mutation (3 available); any Tmc1 mutation (28 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
hearing/vestibular/ear
• degeneration of most elements of the scala media complex is underway by three weeks of age
• Dieters cell boundaries become indistinct and nuclei move to different levels by 10 days of age
• by 9 months of age, the organ is a mass of undifferentiated cells, although the tunnel of Corti does not usually collapse
• abnormalities can be detected at 10 days of age
• the free end of the membrane curls up rather than resting on hair cells by 10 days
• some degeneration is observed in most mice
• in severe cases, hair cells degenerate completely
• in some mice, the otolith membrane degenerates completely and otoliths are lost
• mice do not respond to clicking

behavior/neurological
• tossing movement is observed in some mice although behavior is otherwise normal

nervous system
N
• no abnormal central or peripheral nervous system phenotupes detected
• spiral ganglion degeneration begins at 50 days of age
• by 9 months, degeneration is nearly complete


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
05/17/2016
MGI 6.03
The Jackson Laboratory