Phenotypes Associated with This Genotype

Genotype
MGI:2675255

• Allelic Composition: Ednrb^WS4/Ednrb^WS4
• Genetic Background: involves: BALB/c * MSM

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

Cochlea comparison of wild type and Ednrb^WS4/Ednrb^WS4 mice at 6 weeks of age

mortality/aging

premature death ( J:76584 )

• animals appear sick after two weeks of age and die between 2 and 7 weeks after birth

pigmentation

absent strial intermediate cells ( J:76584 )

variable body spotting ( J:76584 )

• extensive white spotting

digestive/alimentary system

megacolon ( J:76584 )

hearing/vestibular/ear

abnormal cochlea morphology ( J:76584 )

• endolymphatic collapse in the cochleae

cochlear hair cell degeneration ( J:76584 )

organ of Corti degeneration ( J:76584 )

abnormal stria vascularis morphology ( J:76584 )

absent strial intermediate cells ( J:76584 )

thin stria vascularis ( J:76584 )

abnormal hearing physiology ( J:76584 )

deafness ( J:76584 )

nervous system

cochlear hair cell degeneration ( J:76584 )

integument

variable body spotting ( J:76584 )

• extensive white spotting

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Waardenburg syndrome type 4A		DOID:0110953	OMIM:277580	J:76584