Mouse Genome Informatics
hm
    Msrb3tm1Hyk/Msrb3tm1Hyk
involves: 129 * C57BL/6
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
hearing/vestibular/ear
N
• mice exhibit normal vestibular behaviors (J:206322)
• mice exhibit normal protein oxidation in the inner ear cells (J:206322)
• omega-shaped arrangement of stereocilia
• progressive degeneration of stereocilia of the inner ear hair cells beginning at P13, more severe at P20
• degeneration is more severe in the apical turns than in the middle and basal turns
• progressive degeneration of stereocilia of the inner ear hair cells beginning at P13, more severe at P20
• degeneration is more severe in the apical turns than in the middle and basal turns
• mice did not respond to click stimuli or tone bursts

cellular
N
• mice exhibit normal protein oxidation in the inner ear cells (J:206322)
• in the organ of Corti

nervous system
• omega-shaped arrangement of stereocilia
• progressive degeneration of stereocilia of the inner ear hair cells beginning at P13, more severe at P20
• degeneration is more severe in the apical turns than in the middle and basal turns
• progressive degeneration of stereocilia of the inner ear hair cells beginning at P13, more severe at P20
• degeneration is more severe in the apical turns than in the middle and basal turns

Mouse Models of Human Disease
OMIM IDRef(s)
Deafness, Autosomal Recessive 74; DFNB74 613718 J:206322