Phenotypes Associated with This Genotype

Genotype
MGI:5569513

• Allelic Composition: Msrb3^tm1Hyk/Msrb3^tm1Hyk
• Genetic Background: involves: 129 * C57BL/6

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:206322 )

N • mice exhibit normal vestibular behaviors

N • mice exhibit normal protein oxidation in the inner ear cells

abnormal outer hair cell stereociliary bundle morphology ( J:206322 )

• omega-shaped arrangement of stereocilia

absent outer hair cell stereocilia ( J:206322 )

• at P50

cochlear inner hair cell degeneration ( J:206322 )

• 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

cochlear outer hair cell degeneration ( J:206322 )

• 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

increased or absent threshold for auditory brainstem response ( J:206322 )

• absent amplitude

deafness ( J:206322 )

• mice did not respond to click stimuli or tone bursts

cellular

cellular phenotype ( J:206322 )

N • mice exhibit normal protein oxidation in the inner ear cells

increased apoptosis ( J:206322 )

• in the organ of Corti

nervous system

abnormal outer hair cell stereociliary bundle morphology ( J:206322 )

• omega-shaped arrangement of stereocilia

absent outer hair cell stereocilia ( J:206322 )

• at P50

cochlear inner hair cell degeneration ( J:206322 )

• 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

cochlear outer hair cell degeneration ( J:206322 )

• 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		DO ID	OMIM ID(s)	Ref(s)
autosomal recessive nonsyndromic deafness 74		DOID:0110523	OMIM:613718	J:206322