Phenotypes Associated with This Genotype

Genotype
MGI:7571494

• Allelic Composition: Wfs1^tm1.1Bedel/Wfs1^tm1.1Bedel
• Genetic Background: involves: C57BL/6N

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

behavior/neurological

abnormal postural reflex ( J:342760 )

• abnormal behavior in contact-inhibition and tail-hanging tests

• tail-hanging reflex worsening with age between P21 and P31

impaired righting response ( J:342760 )

• worsening with age between P21 and P31

impaired coordination ( J:342760 )

• shorter latency to fall in rotarod test at age P21

impaired swimming ( J:342760 )

• worsening with age between P21 and P31

head bobbing ( J:342760 )

• worsening with age between P21 and P31

circling ( J:342760 )

• worsening with age between P21 and P31

hearing/vestibular/ear

fused inner hair cell stereocilia ( J:342760 )

• by age P31

• normal at age P23

absent outer hair cell stereocilia ( J:342760 )

• by age P31

• normal at age P23

stria vascularis degeneration ( J:342760 )

• at age P31, starting at age P23

abnormal inner ear vestibule morphology ( J:342760 )

• complete loss of vestibular epithelium by age P31

• normal well-organized vestibular epithelium at age P23

fused vestibular hair cell stereocilia ( J:342760 )

vestibular hair cell degeneration ( J:342760 )

• by age P31

decreased endocochlear potential ( J:342760 )

• at age P31

increased or absent threshold for auditory brainstem response ( J:342760 )

• higher ABR threshold starting at high frequencies at age P23 and across all frequencies by age P27-P29

absent distortion product otoacoustic emissions ( J:342760 )

• by age P27-P29

deafness ( J:342760 )

• by age P27-P29

nervous system

nervous system phenotype ( J:342760 )

N • normal spiral ganglion neuron morphology at age P21-23

N • normal spiral ganglion density at age P31

fused inner hair cell stereocilia ( J:342760 )

• by age P31

• normal at age P23

absent outer hair cell stereocilia ( J:342760 )

• by age P31

• normal at age P23

fused vestibular hair cell stereocilia ( J:342760 )

vestibular hair cell degeneration ( J:342760 )

• by age P31

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Wolfram syndrome 1		DOID:0110629	OMIM:222300	J:342760