Phenotypes Associated with This Genotype

Genotype
MGI:6163704

• Allelic Composition: Gt(ROSA)26Sor^{em1(ptxA)Btar}/?; Tg(Atoh1-cre)1Bfri/?
• Genetic Background: B6.Cg-Gt(ROSA)26Sor^{em1(ptxA)Btar} Tg(Atoh1-cre)1Bfri/Btar

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

hearing/vestibular/ear

abnormal cochlear hair cell stereociliary bundle morphology ( J:236519 )

• similar to Gpsm2 null homozygotes, the inner hair cells show supernumerary rows of nearly identical stereocilia with a much shortened first row and lacking the normal staircase pattern of heights

abnormal inner hair cell stereociliary bundle morphology ( J:236519 )

short inner hair cell stereocilia ( J:236519 )

abnormal vestibular hair cell stereociliary bundle morphology ( J:236519 )

• in the utricle the vestibular hair cells also have stereocilia that are shortened and have a shallower staircase pattern

increased or absent threshold for auditory brainstem response ( J:236519 )

• almost completely deaf

deafness ( J:236519 )

nervous system

abnormal cochlear hair cell stereociliary bundle morphology ( J:236519 )

• similar to Gpsm2 null homozygotes, the inner hair cells show supernumerary rows of nearly identical stereocilia with a much shortened first row and lacking the normal staircase pattern of heights

abnormal inner hair cell stereociliary bundle morphology ( J:236519 )

short inner hair cell stereocilia ( J:236519 )

abnormal vestibular hair cell stereociliary bundle morphology ( J:236519 )

• in the utricle the vestibular hair cells also have stereocilia that are shortened and have a shallower staircase pattern