Phenotypes associated with this allele

• Allele Symbol: Tmprss3^m1Ingm
• Allele Name: mutation 1, Ingenium Pharmaceuticals
• Allele ID: MGI:5009322
• Summary: 1 genotype

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Tmprss3^m1Ingm/Tmprss3^m1Ingm	C3HeB/FeJ-Tmprss3^m1Ingm	MGI:5009334

Genotype
MGI:5009334

hm1

• Allelic Composition: Tmprss3^m1Ingm/Tmprss3^m1Ingm
• Genetic Background: C3HeB/FeJ-Tmprss3^m1Ingm

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

Degeneration of hair cells in Tmprss3^m1Ingm/Tmprss3^m1Ingm mice at the onset of hearing

hearing/vestibular/ear

hearing/vestibular/ear phenotype ( J:172676 )

N • mice exhibit normal stria vascularis function

abnormal kinocilium morphology ( J:172676 )

• from birth to P11, mice occasionally have one or two additional thin and long kinocilia on the outer hair cells and inner hair cells located near the thick kinocilium unlike in wild-type mice

abnormal cochlea morphology ( J:172676 )

• at P12 in the basal and middle cochlear turns

abnormal organ of Corti morphology ( J:172676 )

• the tunnel of Corti and spaces of Nuel are collapsed unlike in wild-type mice

absent cochlear hair cells ( J:172676 )

absent cochlear inner hair cells ( J:172676 )

absent cochlear outer hair cells ( J:172676 )

abnormal inner hair cell stereociliary bundle morphology ( J:172676 )

• immature at birth

• however, adult morphology is reached by P11

abnormal outer hair cell stereociliary bundle morphology ( J:172676 )

• immature at birth

• however, adult morphology is reached by P11

cochlear hair cell degeneration ( J:172676 )

• after P11

cochlear inner hair cell degeneration ( J:172676 )

• from P12 onward

cochlear outer hair cell degeneration ( J:172676 )

• from P12 onward

organ of Corti degeneration ( J:172676 )

• regressed to the basal cochlear turn by 4.5 months of age

abnormal stria vascularis morphology ( J:172676 )

• mice exhibit a slight swelling of intercellular spaces between marginal and intermediate cells unlike wild-type mice

absent vestibular hair cells ( J:172676 )

vestibular hair cell degeneration ( J:172676 )

• in the saccule

abnormal vestibular saccule morphology ( J:172676 )

• saccular epithelia is abnormal in cell composition and organization compared with in wild-type mice

• after 4 months, mice lack saccular hair cells unlike wild-type mice

increased or absent threshold for auditory brainstem response ( J:172676 )

absent distortion product otoacoustic emissions ( J:172676 )

deafness ( J:172676 )

• at P60

abnormal vestibular system physiology ( J:172676 )

• mice exhibit increased latency to return on the contact inhibition reflex compared with wild-type mice

• mice exhibit a tendency to occipital landings when landing at the tail hanging reflex unlike wild-type mice

• mice tend to swim to the side unlike wild-type mice

behavior/neurological

impaired coordination ( J:172676 )

• on a rotarod due to vestibular dysfunction

impaired swimming ( J:172676 )

• mice tend to swim to the side unlike wild-type mice

nervous system

abnormal kinocilium morphology ( J:172676 )

• from birth to P11, mice occasionally have one or two additional thin and long kinocilia on the outer hair cells and inner hair cells located near the thick kinocilium unlike in wild-type mice

absent cochlear hair cells ( J:172676 )

absent cochlear inner hair cells ( J:172676 )

absent cochlear outer hair cells ( J:172676 )

abnormal inner hair cell stereociliary bundle morphology ( J:172676 )

• immature at birth

• however, adult morphology is reached by P11

abnormal outer hair cell stereociliary bundle morphology ( J:172676 )

• immature at birth

• however, adult morphology is reached by P11

cochlear hair cell degeneration ( J:172676 )

• after P11

cochlear inner hair cell degeneration ( J:172676 )

• from P12 onward

cochlear outer hair cell degeneration ( J:172676 )

• from P12 onward

absent vestibular hair cells ( J:172676 )

vestibular hair cell degeneration ( J:172676 )

• in the saccule

cochlear ganglion degeneration ( J:172676 )

• at 4 months of age

cellular

abnormal kinocilium morphology ( J:172676 )

• from birth to P11, mice occasionally have one or two additional thin and long kinocilia on the outer hair cells and inner hair cells located near the thick kinocilium unlike in wild-type mice