Phenotypes associated with this allele

• Allele Symbol: Tg(Atoh1-Clrn1)#Kuna
• Allele Name: transgene insertion, Kumar N Alagramam
• Allele ID: MGI:6156639
• Summary: 2 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
cx1	Clrn1^tm2.1Kuna/Clrn1^tm2.1Kuna Tg(Atoh1-Clrn1)#Kuna/0	involves: 129 * C57BL/6J	MGI:6199482
cx2	Clrn1^tm1.1Kuna/Clrn1^tm1.1Kuna Tg(Atoh1-Clrn1)#Kuna/0	involves: C57BL/6J	MGI:6156835

Genotype
MGI:6199482

cx1

• Allelic Composition: Clrn1^tm2.1Kuna/Clrn1^tm2.1Kuna; Tg(Atoh1-Clrn1)#Kuna/0
• Genetic Background: involves: 129 * C57BL/6J

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

hearing/vestibular/ear

increased or absent threshold for auditory brainstem response ( J:260239 )

• by P70, 2 of 10 mice show a 70-80 dB SPL threshold indicating severe hearing loss and 8 of 10 mice show a greater than 90 dB SPL threshold, indicating profound hearing loss

deafness ( J:260239 )

• mice show gradual hearing loss such that at P22, most mice have auditory brain stem response (ABR) thresholds similar to controls but by P70, 2 of 10 mice show a 70-80 dB SPL threshold indicating severe hearing loss and 8 of 10 mice show a greater than 90 dB SPL threshold, indicating profound hearing loss

• treatment with a small molecule BF844 mitigates hearing loss

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Usher syndrome type 3		DOID:0110828		J:260239

Genotype
MGI:6156835

cx2

• Allelic Composition: Clrn1^tm1.1Kuna/Clrn1^tm1.1Kuna; Tg(Atoh1-Clrn1)#Kuna/0
• Genetic Background: involves: C57BL/6J

hearing/vestibular/ear

abnormal inner hair cell stereociliary bundle morphology ( J:255408 )

• progressive deterioration of bundle structure from age P30

• some ears with patches with only few, disrupted, bundles remaining at age P100

• grossly comparable to wild-type at age P2, P10, P21 and P36

abnormal outer hair cell stereociliary bundle morphology ( J:255408 )

• shortest row of stereocilia depleted more than in wild-type at age P21

• progressive deterioration of bundle structure from age P30

• some ears with patches with only few, disrupted, bundles remaining at age P100

• grossly comparable to wild-type at age P2, P10, P21 and P36

impaired hearing ( J:255408 )

• delayed onset progressive hearing loss resulting in deafness by age P70

nervous system

abnormal inner hair cell stereociliary bundle morphology ( J:255408 )

• progressive deterioration of bundle structure from age P30

• some ears with patches with only few, disrupted, bundles remaining at age P100

• grossly comparable to wild-type at age P2, P10, P21 and P36

abnormal outer hair cell stereociliary bundle morphology ( J:255408 )

• shortest row of stereocilia depleted more than in wild-type at age P21

• progressive deterioration of bundle structure from age P30

• some ears with patches with only few, disrupted, bundles remaining at age P100

• grossly comparable to wild-type at age P2, P10, P21 and P36

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Usher syndrome type 3		DOID:0110828		J:255408