Phenotypes Associated with This Genotype

Genotype
MGI:6156835

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

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

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