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Phenotypes Associated with This Genotype
Genotype
MGI:5487460
Allelic
Composition
Myo7admbo2/Myo7admbo2
Genetic
Background
involves: C57BL/6J
Find Mice Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Myo7admbo2 mutation (0 available); any Myo7a mutation (66 available)
phenotype observed in females
phenotype observed in males
N normal phenotype

Degeneration and/or misorientation of outer hair cell bundles in Myo7aewaso/Myo7aewaso and Myo7admbo2/Myo7admbo2 cochlea

hearing/vestibular/ear
N
• mice exhibit normal vestibular function, tympanic membrane, bulla, ossicles, supporting cells and zone of polarity reversal of vestibular hair cell bundles
• progressive degeneration with additional rows of stereocilia
• misoriented at P5 in the apical, mid and basal levels
• more so at 2 weeks at all levels
• progressive misorientation with advancing age with disintegration in parts
• however, sensory epithelium degeneration is minimal at 8 weeks and large numbers of outer hair cell bundles are still present
• some of the tallest or shortest sterocilia are missing in some bundles
• severe from 4 weeks
• however, mice retain some residual hearing at 24 weeks
• severe from 4 weeks

nervous system
• progressive degeneration with additional rows of stereocilia
• misoriented at P5 in the apical, mid and basal levels
• more so at 2 weeks at all levels
• progressive misorientation with advancing age with disintegration in parts
• however, sensory epithelium degeneration is minimal at 8 weeks and large numbers of outer hair cell bundles are still present
• some of the tallest or shortest sterocilia are missing in some bundles

Mouse Models of Human Disease
OMIM ID Ref(s)
Deafness, Autosomal Recessive 2; DFNB2 600060 J:195666


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