Phenotypes associated with this allele

• Allele Symbol: Tecta⁺
• Allele Name: wild type
• Allele ID: MGI:2440345
• Summary: 6 genotypes

Jump to	Allelic Composition	Genetic Background	Genotype ID
ht1	Tecta^tm3.1Gpr/Tecta^+	involves: 129S/SvEv	MGI:5527171
ht2	Tecta^tm4.1Gpr/Tecta^+	involves: 129S/SvEv	MGI:5527173
ht3	Tecta^tm5.1Gpr/Tecta^+	involves: 129S/SvEv	MGI:5527175
ht4	Tecta^tm2Gpr/Tecta^+	involves: 129S/SvEv * C57BL/6J	MGI:3605834
ht5	Tecta^tm1.1Ogha/Tecta^+	involves: 129S4/SvJaeSor * 129S7/SvEvBrd * CBA	MGI:4458443
cx6	Slc26a5^tm1Jnz/Slc26a5^tm1Jnz Tecta^tm1.1Ogha/Tecta^+	involves: 129S7/SvEvBrd * CBA	MGI:4458446

Genotype
MGI:5527171

ht1

• Allelic Composition: Tecta^tm3.1Gpr/Tecta⁺
• Genetic Background: involves: 129S/SvEv

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

hearing/vestibular/ear

abnormal cochlear hair cell stereociliary bundle morphology ( J:203482 )

• reduced hair-bundle attachment with imprints not observed in the apical, low-frequency regions and are only apparent in more basal regions

abnormal Hensen stripe morphology ( J:203482 )

• Hensen's stripe not as prominent as in wild-type mice

• Hensen's stripe lacks typical V-shape

abnormal tectorial membrane morphology ( J:203482 )

• distorted cross-sectional profile with the limbal zone not extending fully across the surface of the spiral limbus in the medial direction, disrupted marginal band, and not as prominent Hensen's stripe as in wild-type mice

• the covernet is hard to discern with reduced fibril diameter with the presence of exceptionally large diameter fibrils

abnormal tectorial membrane covernet morphology ( J:203482 )

abnormal tectorial membrane marginal band morphology ( J:203482 )

• disrupted marginal band

increased or absent threshold for auditory brainstem response ( J:203482 )

• less severe hearing loss than in Tecta^tm4.1Gpr or Tecta^tm5.1Gpr heterozygotes

• however, increase is stable with time

impaired hearing ( J:203482 )

• less severe hearing loss than in Tecta^tm4.1Gpr or Tecta^tm5.1Gpr heterozygotes

behavior/neurological

audiogenic seizures ( J:203482 )

• preceded by wild running

nervous system

audiogenic seizures ( J:203482 )

• preceded by wild running

abnormal cochlear hair cell stereociliary bundle morphology ( J:203482 )

• reduced hair-bundle attachment with imprints not observed in the apical, low-frequency regions and are only apparent in more basal regions

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal dominant nonsyndromic deafness 12		DOID:0110544	OMIM:601543	J:203482

Genotype
MGI:5527173

ht2

• Allelic Composition: Tecta^tm4.1Gpr/Tecta⁺
• Genetic Background: involves: 129S/SvEv

hearing/vestibular/ear

abnormal cochlear hair cell stereociliary bundle morphology ( J:203482 )

• reduced hair-bundle attachment with imprints only apparent in apical, lower-frequency regions

abnormal Hensen stripe morphology ( J:203482 )

• Hensen's stripe is no longer attached directly to the tectorial membrane

abnormal tectorial membrane morphology ( J:203482 )

• hump-backed tectorial membrane with reduced extent of the limbal zone and medially displaced marginal band

• most apparent changes are in the cross-sectional profile and the extent of limbal attachment

• the dense matrix is mostly missing from the severely reduced limbal zone

• collagen fibrils erupt form the upper surface of the tectorial membrane in the inner sulcal region and occasionally extend out from the lower surface in the sulcal region immediately adjacent to the limbal attachment point

abnormal Kimura membrane morphology ( J:203482 )

• Kimura's membrane is delaminated and fenestrated

abnormal tectorial membrane covernet morphology ( J:203482 )

• covernet fibrils are reduced in numbers, more convoluted and run both longitudinally and radially across the surface of the tectorial membrane compared to in wild-type mice

• covernet fibrils in the sulcal and more medial regions are less tightly associated with the main body of the tectorial membrane

abnormal tectorial membrane striated-sheet matrix morphology ( J:203482 )

• the striated-sheet matrix is restricted to the lateral part of the tectorial membrane

increased or absent threshold for auditory brainstem response ( J:203482 )

• intermediate hearing loss between that observed in Tecta^tm3.1Gpr and Tecta^tm5.1Gpr heterozygotes

impaired hearing ( J:203482 )

• intermediate hearing loss between that observed in Tecta^tm3.1Gpr and Tecta^tm5.1Gpr heterozygotes

• however, increase is stable with time

behavior/neurological

audiogenic seizures ( J:203482 )

• preceded by wild running

nervous system

audiogenic seizures ( J:203482 )

• preceded by wild running

abnormal cochlear hair cell stereociliary bundle morphology ( J:203482 )

• reduced hair-bundle attachment with imprints only apparent in apical, lower-frequency regions

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal dominant nonsyndromic deafness 12		DOID:0110544	OMIM:601543	J:203482

Genotype
MGI:5527175

ht3

• Allelic Composition: Tecta^tm5.1Gpr/Tecta⁺
• Genetic Background: involves: 129S/SvEv

hearing/vestibular/ear

abnormal cochlear hair cell stereociliary bundle morphology ( J:203482 )

• reduced hair-bundle attachment with imprints only apparent in apical, lower-frequency regions

abnormal Hensen stripe morphology ( J:203482 )

• Hensen's stripe is no longer attached directly to the tectorial membrane

abnormal tectorial membrane morphology ( J:203482 )

• hump-backed tectorial membrane with reduced extent of the limbal zone and medially displaced marginal band

• most apparent changes are in the cross-sectional profile and the extent of limbal attachment

• the dense matrix is mostly missing from the severely reduced limbal zone

• collagen fibrils erupt form the upper surface of the tectorial membrane in the inner sulcal region and occasionally extend out from the lower surface in the sulcal region immediately adjacent to the limbal attachment point

abnormal Kimura membrane morphology ( J:203482 )

• Kimura's membrane is delaminated and fenestrated

abnormal tectorial membrane covernet morphology ( J:203482 )

• covernet fibrils are reduced in numbers, more convoluted and run both longitudinally and radially across the surface of the tectorial membrane compared to in wild-type mice

• covernet fibrils in the sulcal and more medial regions are less tightly associated with the main body of the tectorial membrane

abnormal tectorial membrane striated-sheet matrix morphology ( J:203482 )

• the striated-sheet matrix is restricted to the lateral part of the tectorial membrane

increased or absent threshold for auditory brainstem response ( J:203482 )

• more severe hearing loss than in Tecta^tm3.1Gpr or Tecta^tm4.1Gpr heterozygotes

impaired hearing ( J:203482 )

• more severe hearing loss than in Tecta^tm3.1Gpr or Tecta^tm4.1Gpr heterozygotes

• however, increase is stable with time

behavior/neurological

audiogenic seizures ( J:203482 )

• preceded by wild running

nervous system

audiogenic seizures ( J:203482 )

• preceded by wild running

abnormal cochlear hair cell stereociliary bundle morphology ( J:203482 )

• reduced hair-bundle attachment with imprints only apparent in apical, lower-frequency regions

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
autosomal dominant nonsyndromic deafness 12		DOID:0110544	OMIM:601543	J:203482

Genotype
MGI:3605834

ht4

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

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

Genotype
MGI:4458443

ht5

• Allelic Composition: Tecta^tm1.1Ogha/Tecta⁺
• Genetic Background: involves: 129S4/SvJaeSor * 129S7/SvEvBrd * CBA

hearing/vestibular/ear

abnormal tectorial membrane morphology ( J:160851 )

• the tectorial membrane is thicker and shorter than in wild-type mice but not as much as in homozygous mice

• the fibrils surrounding the edge of the tectorial membrane are partially disrupted compared to in wild-type mice

abnormal Kimura membrane morphology ( J:160851 )

• Kimura's membrane contains loosely packed fibrils compared to in wild-type mice

abnormal cochlear outer hair cell physiology ( J:160851 )

• during acoustic stimulation of the stapes, only outer hair cells in the first row exhibit forward transduction unlike in similarly treated wild-type cells

abnormal cochlear microphonics ( J:160851 )

• in response to an increasing intensity of accoustic stimuli, mice exhibit an increase in cochlear microphonic phase lead unlike in similarly treated wild-type mice

increased or absent threshold for auditory brainstem response ( J:160851 )

• heterozygous mice had 25-40 dB ABR threshold elevations relative to wild-type littermates

abnormal otoacoustic response ( J:160851 )

• mice exhibit enhanced reverse transduction compared with wild-type mice

abnormal distortion product otoacoustic emission ( J:160851 )

• distortion product otoacoustic emission exhibit elevated thresholds, reduced amplitudes, and higher slopes compared to in wild-type mice

impaired hearing ( J:160851 )

• mice exhibit partial hearing loss compared with wild-type mice

nervous system

abnormal cochlear outer hair cell physiology ( J:160851 )

• during acoustic stimulation of the stapes, only outer hair cells in the first row exhibit forward transduction unlike in similarly treated wild-type cells

abnormal cochlear microphonics ( J:160851 )

• in response to an increasing intensity of accoustic stimuli, mice exhibit an increase in cochlear microphonic phase lead unlike in similarly treated wild-type mice

Genotype
MGI:4458446

cx6

• Allelic Composition: Slc26a5^tm1Jnz/Slc26a5^tm1Jnz; Tecta^tm1.1Ogha/Tecta⁺
• Genetic Background: involves: 129S7/SvEvBrd * CBA

hearing/vestibular/ear

abnormal otoacoustic response ( J:160851 )

• electrically evoked otoacoustic emissions amplitudes are nearly completely lost unlike in wild-type mice