Phenotypes Associated with This Genotype

Genotype
MGI:3605834

• Allelic Composition: Tecta^tm2Gpr/Tecta⁺
• Genetic Background: involves: 129S/SvEv * C57BL/6J

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

hearing/vestibular/ear

absent Hensen stripe ( J:101691 )

• Hensen's stripe is absent

abnormal tectorial membrane morphology ( J:101691 )

• limbal zone of tectorial membrane is extremely reduced, however hair bundle structure is normal

• enlargement of the subtectorial space in the vicinity of inner hair cell bundles

abnormal Kimura membrane morphology ( J:101691 )

• Kimura's membrane is separated from the main body of the tectorial membrane

abnormal tectorial membrane marginal band morphology ( J:101691 )

• the marginal band is absent

abnormal tectorial membrane striated-sheet matrix morphology ( J:101691 )

• large holes are visible within the main body of the matrix

• striated-sheet matrix is not present in the sulcal zone and collagen fibrils are oriented almost perpendicular to its surface rather than projecting radially across the tectorial membrane

abnormal hearing physiology ( J:101691 )

• neural response thresholds are elevated, neural tuning is broadened, and a sharp decrease in sensitivity is observed at the tip of the neural tuning curve, however the sensitivity and frequency tuning of the mechanical responses of the cochlea are little changed

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal dominant nonsyndromic deafness 12		DOID:0110544	OMIM:601543	J:101691