Phenotypes associated with this allele

• Allele Symbol: Atp2b2^Obv
• Allele Name: oblivion
• Allele ID: MGI:4355879
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
hm1	Atp2b2^Obv/Atp2b2^Obv	C3HeB/FeJ-Atp2b2^Obv	MGI:4355897
ht2	Atp2b2^Obv/Atp2b2^+	C3HeB/FeJ-Atp2b2^Obv	MGI:4355896

Genotype
MGI:4355897

hm1

• Allelic Composition: Atp2b2^Obv/Atp2b2^Obv
• Genetic Background: C3HeB/FeJ-Atp2b2^Obv

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

hearing/vestibular/ear

cochlear hair cell degeneration ( J:151733 )

• at P30, mice exhibit highly variable hair cell degeneration unlike in wild-type mice

• hair cells completely degenerate from the basal turn of the cochlea

• however, hair cells in the apex of the cochlea appear normal

pillar cell degeneration ( J:151733 )

• in some mice

organ of Corti degeneration ( J:151733 )

• in some mice, the organ of Corti is degenerated along with all specialized cells such as pillar cells

abnormal vestibular hair cell physiology ( J:151733 )

• dissipation of calcium ion transients in utricular hair cells is slower than in wild-type cells

deafness ( J:151733 )

• mice are death from birth

abnormal vestibular system physiology ( J:151733 )

• mice exhibit severe vestibular dysfunction as early as 2 weeks of age

behavior/neurological

abnormal reflex ( J:151733 )

• when suspended by their tail, mice curl towards their belly and do not display a reaching response when lowered towards the surface unlike similarly treated wild-type mice

• mice display hindlimb stiffness and splaying

absent pinna reflex ( J:151733 )

• mice fail to develop a Preyer reflex

impaired righting response ( J:151733 )

• mice are unable to right themselves

ataxia ( J:151733 )

impaired balance ( J:151733 )

• mice fall while walking

head tossing ( J:151733 )

circling ( J:151733 )

• in some mice

growth/size/body

decreased body size ( J:151733 )

decreased body weight ( J:151733 )

nervous system

cochlear hair cell degeneration ( J:151733 )

• at P30, mice exhibit highly variable hair cell degeneration unlike in wild-type mice

• hair cells completely degenerate from the basal turn of the cochlea

• however, hair cells in the apex of the cochlea appear normal

abnormal vestibular hair cell physiology ( J:151733 )

• dissipation of calcium ion transients in utricular hair cells is slower than in wild-type cells

Genotype
MGI:4355896

ht2

• Allelic Composition: Atp2b2^Obv/Atp2b2⁺
• Genetic Background: C3HeB/FeJ-Atp2b2^Obv

hearing/vestibular/ear

cochlear hair cell degeneration ( J:151733 )

• at 3 to 4 months, mice exhibit degeneration of hair cells with the basal turn more severely affected than the apex and outer hair cells more severely affected than inner hair cells unlike in wild-type mice

• loss of hair cells occurs secondary to loss of hearing

cochlear inner hair cell degeneration ( J:151733 )

• at P121, inner hair cell loss is observed at the base of the cochlea

cochlear outer hair cell degeneration ( J:151733 )

• at P75, outer hair cell loss is prominent in the base but not the middle of the cochlea

• at P121, outer hair cell loss is observed in the base and middle of the cochlea

abnormal vestibular hair cell physiology ( J:151733 )

• dissipation of calcium ion transients in utricular hair cells is slower than in wild-type cells

increased or absent threshold for auditory brainstem response ( J:151733 )

• at early as P20, brainstem auditory evoked potential (ABR) thresholds are increased at all frequencies tested compared to in wild-type mice

• at P59 to P62, thresholds are further increased compared to in wild-type mice

impaired hearing ( J:151733 )

• mice exhibit severe and age-related progressive hearing loss

deafness ( J:151733 )

• most mice are deaf by 2 months of age

behavior/neurological

absent pinna reflex ( J:151733 )

• at 2 months, 42% of mice lack a normal Preyer reflex

• at 3 months, no mice display a normal Preyer reflex

• however, the Preyer reflex is normal at 1 month

nervous system

cochlear hair cell degeneration ( J:151733 )

• at 3 to 4 months, mice exhibit degeneration of hair cells with the basal turn more severely affected than the apex and outer hair cells more severely affected than inner hair cells unlike in wild-type mice

• loss of hair cells occurs secondary to loss of hearing

cochlear inner hair cell degeneration ( J:151733 )

• at P121, inner hair cell loss is observed at the base of the cochlea

cochlear outer hair cell degeneration ( J:151733 )

• at P75, outer hair cell loss is prominent in the base but not the middle of the cochlea

• at P121, outer hair cell loss is observed in the base and middle of the cochlea

abnormal vestibular hair cell physiology ( J:151733 )

• dissipation of calcium ion transients in utricular hair cells is slower than in wild-type cells